Problem Solving In Rheumatology


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Problem Solving In Rheumatology

  1. 1. Problem Solving in Rheumatology KEVIN PILE MB ChB, MD, FRACP Conjoint Professor of Medicine, University of Western Sydney, New South Wales, Australia LEE KENNEDY BSc, MB ChB, MD, PhD, FRCP, FRCPE, FRACP Professor of Medicine, School of Medicine, Department of Medicine, James Cook University, Queensland, Australia CLINICAL PUBLISHING OXFORD
  2. 2. Contents Abbreviations vii SECTION 1 General Rheumatology and Soft Tissue Rheumatism 1. New Onset Painful Joints 1 2. An Acutely Swollen/Hot Joint 6 3. Painful Shoulders – Rotator Cuff and Frozen Shoulder 11 4. Tennis Elbow and Golfer’s Elbow 18 5. Carpal Tunnel Syndrome and Other Entrapment Neuropathies 21 6. Fibromyalgia Syndrome 27 7. Plantar Fasciitis 33 SECTION 2 Osteoarthritis 8. Causes and Prevention 39 9. Non-Pharmacological Treatment 45 10. Drug Treatment 50 11. NSAIDs – Gastric Side Effects and Protection 54 12. NSAIDs – Cardiac Complications 60 13. Joint Replacement Surgery 65 SECTION 3 Rheumatoid Arthritis 14. Causes 71 15. Laboratory and Imaging Investigations 77 16. Managing Rheumatoid Arthritis at Onset 82 17. Evaluating the Response to Treatment 87 18. Pregnancy and Rheumatic Diseases 92 19. Diet and Arthritis 97 20. Polyarthritis in the Elderly 103 SECTION 4 Systemic Lupus Erythematosus, Sjögren’s Syndrome and Scleroderma 21. Antinuclear Factor 109 22. SLE – Risk Factors and Diagnosis 116 23. Monitoring and Managing SLE 122
  3. 3. vi Contents 24. Sjögren’s Syndrome 129 25. Raynaud’s Phenomenon 134 26. Assessing and Treating Scleroderma 139 27. Immunosuppressive Drugs 147 SECTION 5 Vasculitic Syndromes 28. Vasculitic Disease 153 29. Giant Cell Arteritis and Polymyalgia Rheumatica 159 30. Behçet’s Syndrome 165 SECTION 6 Back and Specific Joint Problems 31. Acute Back Pain 169 32. Chronic Back Pain 175 33. Psoriatic Arthritis 178 34. Asymptomatic Hyperuricaemia 184 35. Gout – Acute Attack and Beyond 189 36. Pseudogout – Investigation and Management 195 37. Joint and Bone Infections 199 38. Viral Arthritis 205 39. Rheumatological Complications of Diabetes 211 SECTION 7 Bone Diseases 40. Osteoporosis – Prevention and Lifestyle Management 217 41. Bisphosphonates for Osteoporosis – Which Agent and When? 222 42. Osteoporosis – Drugs Other Than Bisphosphonates 227 43. Male Osteoporosis 233 44. Glucocorticoid-Induced Osteoporosis 237 45. Paget’s Disease of Bone 241 46. Bone Complications of Renal Disease 246 SECTION 8 Muscle Diseases 47. Steroid myopathy 253 48. Inflammatory Myopathies 260 49. Muscle Complications of Statin Therapy 265 General index 271
  4. 4. Abbreviations ABD adynamic bone disease CIM critical iIlness myopathy ACE angiotensin-converting enzyme CK creatine kinase ACR American College of Rheumatology CKD chronic kidney disease ADAMTS a disintegrin and metalloproteinase CKD-MBD CKD-mineral and bone disorder with thrombospondin motif CLASS Celecoxib Long-term Arthritis ADFR Activate, Decrease osteoclast Safety Study activity, Free of treatment and Clc-l chloride channel Repeat CMC carpometacarpophalangeal ADP adenosine diphosphate CNS central nervous system ADR adverse drug reaction CORE Continuing Outcomes Relevant to AMP adenosine monophosphate Evista ANA antinuclear antibody COX cyclooxygenase ANCA anti-neutrophil cytoplasmic COX-1 cyclooxygenase-1 antibodies COX-2 cyclooxygenase-2 ANF antinuclear factor CPEO Chronic Progressive External AP alkaline phosphatase Ophthalmoplegia AP-1 activator protein-1 CPPD calcium pyrophosphate dihydrate APPROVe Adenomatous Polyp Prevention on CREST Calcinosis; Raynaud’s phenomenon; Vioxx study Esophageal dysmotility; APS antiphospholipid syndrome Sclerodactyly, Telangiectasia AS ankylosing spondylitis CRP C-reactive protein ASC apoptosis-associated speck-like CSS Churg–Strauss syndrome protein CT computed tomography ATP adenosine triphosphate CTG cytosine-thymine-guanine B19 parvovirus B19 CTGF connective tissue growth factor BASMI British Ankylosing Spondylitis CTS carpal tunnel syndrome Metrology Index CTLA4-Ig cytotoxic lymphocyte-associated BMD bone mineral density antigen linked to immunoglobulin BMI body mass index CVD cardiovascular disease BP blood pressure CXR chest X-ray BPs bisphosphonates D3 1,25-dihydroxy-vitamin D3 C5 fifth cervical segment DC dendritic cell c-ANCA cytoplasmic anti-neutrophil DD Dupuytren’s disease cytoplasmic antibody DEXA dual-energy X-ray absorptiometry CCB calcium channel blocker DHA docosahexaenoic acid CCTG cytosine-cytosine-thymine-guanine DHEA dehydroepiandrosterone CCL2 monocyte chemoattractant protein- DIL drug-induced lupus 1 (see also MCP-1) DIP distal interphalangeal CCP cyclic citrullinated peptide DISH diffuse idiopathic skeletal CDSN corneodesmin hyperostosis CEP circulating endothelial precursor DLCO diffusing capacity for carbon cGMP cyclic guanosine monophosphate monoxide CHB congenital heart block DM dermatomyositis CI confidence interval DM1 myotonic dystrophy type 1
  5. 5. viii Abbreviations DM2 myotonic dystrophy type 2 hnRNP heterogeneous nuclear DMARD disease-modifying antirheumatic ribonucleoprotein drug HPRT hypoxanthine DMOAD disease-modifying osteoarthritis phosphoribosyltransferase drug HRCT high-resolution computed DMPK myotonic dystrophy protein kinase tomography dsDNA double-stranded DNA HRT hormone replacement therapy EBV Epstein–Barr virus HSP Henoch-Schönlein purpura EDTA ethylenediaminetetraacetic acid HTLV-1 human T-lymphotropic virus type 1 EEG electroencephalogram IBD inflammatory bowel disease EGF epidermal growth factor IBM inclusion body myositis eGFR estimated glomerular filtration rate IFN interferon ELISA enzyme-linked immunosorbent Ig immunoglobulin assay IGF-1 insulin-like growth factor-1 EMG electromyography Iκβ inhibitor of kappa-beta ENA extractable nuclear antigen IL interleukin eNOS endothelial nitric oxide synthase IL-1ra interleukin-1 receptor antagonist EPA eicosapentaenoic acid IMPDH inosine monophosphate ESR erythrocyte sedimentation rate dehydrogenase ET endothelin IMT intima-media thickness FA fatty acid INR International Normalized Ratio FBC full blood count IP inflammatory polyarthritis FDG-PET (18)-F-fluorodeoxyglucose-positron IU International Units emission tomography JSN joint space narrowing FGF fibroblast growth factor LBP low back pain FKBP-12 12 kDa FK506-binding protein LDL low-density lipoprotein FMS fibromyalgia syndrome LFA-1 lymphocyte function-associated FVC forced vital capacity antigen-1 FSH follicle-stimulating hormone LFT liver function test GAIT Glucosamine/chondroitin Arthritis LIFE Losartan Intervention for Endpoint Intervention Trial reduction GCA giant cell arteritis LJM limited joint mobility GDM gestational diabetes LORA late-onset RA GFR glomerular filtration rate LRP-5 LDL receptor-related protein-5 GI gastrointestinal LUMINA Lupus in minorities: nature versus GMP guanosine monophosphate nurture GSD glycogen storage disease LH luteinizing hormone GTP guanosine triphosphate MCP metacarpophalangeal GVHD graft-versus-host disease MCP-1 monocyte chemoattractant protein- H2RA histamine H2 receptor antagonist 1 (see also CCL2) HBA1C glycosylated haemoglobin MCTD mixed connective tissue disease HBO2 hyperbaric oxygen MELAS Myopathy, Encephalopathy, Lactic HDL high-density lipoprotein Acidosis and Stroke HELLP Haemolytic anaemia, Elevated Liver MERRF Myoclonic Epilepsy with Ragged enzymes, Low Platelets Red Fibres HIV human immunodeficiency virus MI myocardial infarction HLA human leukocyte antigen (genetic MMF mycophenolate mofetil designation for human major MMP matrix metalloproteinase histocompatibility complex) MORE Multiple Outcome of Raloxifene HNPP hereditary neuropathy with liability Evaluation to pressure palsies MPA microscopic polyangiitis
  6. 6. Abbreviations ix MRI magnetic resonance imaging PPI proton pump inhibitor MRSA methicillin-resistant Staphylococcus PPRP 5¢phosphoribosyl 1-pyrophosphate aureus PRIMO Prediction of Muscular Risk in MSA myositis-specific antibodies Observational conditions MTOR mammalian target of rapamycin PsA psoriatic arthritis MTP metatarsophalangeal PTH parathyroid hormone MUA manipulation under anaesthesia PTNP22 protein tyrosine phosphate non- NALP pyrin domain-containing proteins receptor type 22 sharing structural homology with PUFAs polyunsaturated fatty acids NODs QALY quality-adjusted life year NCS nerve conduction studies RA rheumatoid arthritis NFAT nuclear factor of activated T RANK receptor activator of NF-κB lymphocytes RANKL receptor activator of NF-κB ligand NF-κB nuclear factor-κ-beta RCT randomized controlled trial NHANES National Health and Nutrition REM rapid eye movement Examination Survey RF rheumatoid factor NIH National Institutes of Health RISC RNA-induced silencing complex NO nitric oxide RNA ribonucleic acid NOD nucleotide-binding and RNP ribonucleoprotein oligomerization domain proteins ROD renal osteodystrophy NOS nitric oxide synthase ROS reactive oxygen species NOS-2 inducible nitric oxide synthase RR relative risk NOS-3 endothelial nitric oxide synthase RS3PE remitting seronegative symmetric (eNOS) synovitis with pitting oedema NSAID non-steroidal anti-inflammatory RUTH Raloxifene Use for The Heart drug SAPHO Synovitis, Acne, Pustulosis, OA osteoarthritis Hyperostosis and Osteitis OCP oral contraceptive pill SE shared epitope 25(OH)D 25-hydroxy-vitamin D SELENA Safety of Estrogens in Lupus OPG osteoprotegerin Erythematosus National Assessment OR odds ratio SERM selective oestrogen receptor PADAM partial androgen deficiency in aging modulator men SHBG sex hormone binding globulin PADI peptidylarginine deaminase SI sacroiliac PAH pulmonary artery hypertension sIL-6R soluble receptor for IL-6 PAN polyarteritis nodosa SJC swollen joint count p-ANCA perinuclear anti-neutrophil SLC22A4 solute carrier family 22 A4 cytoplasmic antibody SLE systemic lupus erythematosus PCR polymerase chain reaction Sm Smith antigen PCT plasma procalcitonin SOBOE shortness of breath on exertion PDGF platelet-derived growth factor SOTI Spinal Osteoporosis Therapeutic PET positron emission tomography Intervention PG prostaglandin SPARC secreted protein acidic and rich in PGI2 prostacyclin cysteine PIP proximal interphalangeal SPECT single photon emission computed PM polymyositis tomography PM/DM polymyositis/dermatomyositis SRP signal recognition particle PMR polymyalgia rheumatica SRRR sibling recurrence risk ratio PP pyrophosphate SS Sjögren’s syndrome PPAR peroxisomal proliferator-activated SSc systemic sclerosis receptor ssDNA single-stranded DNA
  7. 7. x Abbreviations STAT1 signal transducer and activator of TROPOS Treatment Of Peripheral transcription-1 Osteoporosis Study sTNFR soluble receptor for TNF TSH thyroid-stimulating hormone SSRI selective serotonin reuptake TxA2 thromboxane A2 inhibitor U1RNP uracil-rich 1 ribonucleoprotein TB tuberculosis UA uric acid TBF thermal biofeedback U/E urea and electrolytes TGF-β transforming growth factor-β UDP uridine diphosphate Th1 T helper 1 cells UK United Kingdom Th2 T helper 2 cells US United States TIMP tissue inhibitor of UV ultraviolet light metalloproteinase VDR vitamin D receptor TJC tender joint count VEGF vascular endothelial growth factor TLR Toll-like receptor VIGOR Vioxx Gastrointestinal Outcomes TKA total knee arthroplasty Research study TMV turnover, mineralization and WBC white blood cell volume WHO World Health Organization TNF tumour necrosis factor WOMAC Western Ontario and McMaster TNFR2 TNF-α receptor type 2 Universities TRAP tartrate-resistant acid phosphatase XO xanthine oxidase
  8. 8. S E C T I O N O N E 01 General Rheumatology and Soft Tissue Rheumatism 01 New onset painful joints 02 An acutely swollen/hot joint 03 Painful shoulders – rotator cuff and frozen shoulder 04 Tennis elbow and golfer’s elbow 05 Carpal tunnel syndrome and other entrapment neuropathies 06 Fibromyalgia syndrome 07 Plantar fasciitis P R O B L E M 01 New Onset Painful Joints Case History June is a 32-year-old tour guide with an eight-week history of painful stiff hands and difficulty walking in the mornings. The symptoms usually last for 90 minutes. For the last six weeks she has been using diclofenac 50 mg bd with moderate benefit. Her mother has rheumatoid arthritis treated with methotrexate. What additional history will help to determine a diagnosis? What is the relevance of her family history? What aspects of the examination will be particularly relevant? Which investigations should be performed? © Atlas Medical Publishing Ltd
  9. 9. 2 §01 General Rheumatology and Soft Tissue Rheumatism Background History Obtaining a clear history of June’s symptoms will assist greatly in narrowing your initial differential diagnosis as a prelude to examination and investigations. Open questions that encourage the person to start with their initial symptoms provide chronology and the pattern of progression. Gentle prompting can, towards the end of consultation, be supplemented with specific questions. As you listen to the story, you will be assessing the impact of the symptoms on the individual’s life and its components of family, work and leisure. Specifically: b Are symptoms related to a musculoskeletal problem? b Was there an identified trigger or precipitant? b What has been the pattern or progression of symptoms? b Are there features of systemic illness or inflammatory disease? b Has anything helped the problem? Pain and loss of function are primary presenting symptoms, but do not always coexist. Individuals differ in their descriptors of pain, its intensity and its impact. You will be told when the problem began and where. Is the pain in a joint; in a related joint structure such as tendon, ligament or bursa; or in a bone? What is the nature of the pain; when does it occur; and what is the effect of movement? Malignant pain is usually a dull, deep ache within a bone, occurring at night or when resting. Similar symptoms may occur with Paget’s disease or with a fracture. Differentiators of inflammatory from non-inflamma- tory/mechanical joint pain are summarized in Table 1.1. Table 1.1 Differentiators of joint pain Inflammatory pain Non-inflammatory/mechanical pain • Pain and stiffness predominant in morning and at end of day • Short-lived joint stiffness • Stiffness greater than 30 minutes • Pain worsens with activity • Symptoms lessen with activity • Pain improves with rest • Pain does not improve with rest • Localized erythema, swelling, tenderness • Systemic features – fatigue, weight loss Localization of pain requires clarification as to whether symptoms are recreated by contact or movement in the area, or whether the pain is referred from another site. Referred pain occurs when sensory perception externalizes nociceptive input from the sclerotome or myotome of an affected structure to the relevant dermatome. Table 1.2 shows common referred pain patterns. Onset of symptoms following trauma supports mechanical disruption of a joint, dis- ruption of a joint’s surrounding capsule and ligaments, or fracture. Less obvious triggers to explore are infections (Table 1.3), vaccinations (Rubella) and recent travel. A tactful approach is required when soliciting information on genitourinary symptoms or a
  10. 10. 01 New onset painful joints 3 Table 1.2 Common presentations of referred pain Area pain experienced Origin of pain Shoulder Cervical spine Biceps and lateral upper arm Shoulder and rotator cuff Groin, inner knee Hip Lateral thigh, buttock Trochanteric bursa Table 1.3 Common infections associated with arthritis Viral Gastrointestinal Genitourinary Hepatitis B, C Salmonella typhimurium Chlamydia trachomatis Rubella Shigella flexneri Parvovirus Yersinia enterocolitica Arbovirus * Campylobacter jejuni * Serology should be tested according to exposure. history of a new sexual partner, as it is not obvious to a patient with arthritis as to why you would be asking such questions. A comprehensive family history is a key part of every clinical history. A familial pat- tern of a specific diagnosis such as rheumatoid arthritis (RA), ankylosing spondylitis or systemic lupus erythematosus (SLE) highlights that diagnosis, and may also raise related diagnoses that are particularly relevant for seronegative spondyloarthritides such as pso- riasis or inflammatory bowel disease. Examination Examination identifies the pattern and number of joints involved and extra-articular fea- tures (Table 1.4). Features of inflammation are sought: temperature, pulse and blood pressure are measured, and an assessment is made of localized erythema and warmth, tenderness, inflammation obscuring the joint margins, and reduced function. You should distinguish monoarthritis from oligoarthritis (≤4 joints) and polyarthritis (>4 joints), whether these joints are large or small, and whether there is spinal (particularly sacroiliac) involvement. Distal to the wrist and ankle there are at least 56 joints, so that as the number of joints increases, the greater the probability is of involvement of both hands and feet and of the pattern becoming increasingly ‘symmetrical’. Fingernails are assessed for pitting or onycholysis suggestive of psoriasis. The scalp, umbilicus, natal cleft and extensor surfaces of knee and elbow should be inspected. The presence of a malar rash or photosensitive rash in a young woman suggests SLE. Investigations Investigations serve to: b Confirm or refute a diagnostic possibility
  11. 11. 4 §01 General Rheumatology and Soft Tissue Rheumatism Table 1.4 Patterns of arthritis Pattern Monoarthritis Inflammatory Asymmetrical Symmetrical small DIP hands spinal disease large joint joint arthritis Sacroiliitis arthritis (MCP, PIP, MTP) Differential Trauma Ankylosing Psoriatic arthritis RA Inflammatory OA diagnosis spondylitis (if involves PIP and 1st CMC) Haemophilia Psoriatic arthritis Reactive arthritis SLE Psoriatic arthritis Septic IBD IBD Psoriatic arthritis Gout Pseudogout Further X-ray Review personal Review personal Examine X-ray hands investigations and family history and family history rheumatoid nodules Aspirate for HLA-B27 Examine for Skin rashes, crystals and conjunctivitis and serositis or culture urethritis, and scalp mucositis and buttocks for psoriasis X-ray lumbar Infection screen Urinalysis spine and SI joints RF, CCP antibodies, ANA X-ray hands and feet ANA, antinuclear antibodies; CCP, cyclic citrullinated peptides; CMC, carpometacarpophalangeal; DIP, distal interphalangeal; IBD, inflammatory bowel disease; MCP, metacarpophalangeal; MTP, metatarsophalangeal; OA, osteoarthritis; PIP, proximal interphalangeal; RA, rheumatoid arthritis; RF, rheumatoid factor; SI, sacroiliac; SLE, systemic lupus erythematosus. b Monitor for known complications of the disease process or proposed treatment b Document a parameter that changes with disease activity or treatment The latter includes the inflammatory markers erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), which are non-specific markers. Whenever the possibility of a septic joint is considered, obtaining aspirate and culture from the joint is mandatory. Aspirated fluid is collected into a sterile container and an ethylenediaminetetraacetic acid (EDTA)-containing tube to enable a cell count, and is sent with a request for Gram stain- ing, polarized light microscopy, culture and sensitivity, and cell count and differential cell count. If there will be a significant delay in the sample reaching the laboratory, fluid can be inoculated into a blood culture system. The early signs and symptoms of RA are not always typical. RA is characterized as autoimmune partly on the basis of the presence of rheumatoid factor (RF), an autoanti- body (usually immunoglobulin M [IgM]) targeting the Fc portion of IgG. Its sensitivity is low, ranging from 60%–80%, and specificity is lower, the antibody being frequently pre- sent in other connective tissue diseases, which limits the diagnostic utility. Recent Developments 1 RF is present in 70% of RA cases but is not specific, occurring in 5% of healthy individuals, and globally is more associated with chronic infection than rheumatic diseases. Non-RF antibodies were first described in the 1960s, with the target
  12. 12. 01 New onset painful joints 5 epitopes now identified as citrulline residues, which are arginine residues modified by peptidylarginine deaminase (PADI). Assays are now available for the detection of antibodies to cyclic citrullinated peptides (anti-CCP antibodies), which are highly sensitive and specific for RA and are a poor prognostic marker of joint erosion, vasculitis and rheumatoid nodules.1 The specificity of anti-CCP in RA is >90% with sensitivity of 33%–87%. When combined with IgM-RF, anti- CCP has positive predictive value of >90% for RA.2 A study of undifferentiated polyarthritis found that 93% of subjects positive for anti-CCP at first clinic visit progressed to RA compared to 25% who were anti-CCP negative.3 2 Smoking increases the risk of RA 2–4 fold and also influences the manifestations of the disease – with increased RF positivity and erosive disease, nodularity and vasculitis – similar to the findings noted with anti-CCP antibodies. Smoking may break immune tolerance by creating neo-epitopes on IgG and thus leading to RF development. Recent work has shown that smoking is associated with increased citrullination. The subsequent citrullinated antigens bind with more affinity to the HLA-DR4 shared epitope subtypes, leading to increased risk of RA.4 Conclusion Persistent arthropathy in a younger patient necessitates both accurate diagnosis and effective management. A working knowledge of local infectious triggers is required, with supplemental knowledge of the likely pathologies based on age and gender. History and examination need to include potential exposure to infectious triggers, along with per- sonal and family history. Examination will confirm or exclude significant joint inflam- mation, and provide information on its pattern and severity (number of joints and functional impact). Targeted investigations will narrow the diagnosis, with the urgent investigation being exclusion of septic arthritis if there is clinical suspicion. Further Reading 1 Mimori T. Clinical significance of anti-CCP antibodies in rheumatoid arthritis. Intern Med 2005; 44: 1122–6. 2 Schellekens GA, Visser H, De Jong BAW et al. The diagnostic properties of rheumatoid arthri- tis antibodies recognizing a cyclic citrullinated peptide. Arthritis Rheum 2000; 43: 155–63. 3 van Gaalen FA, Linn-Rasker SP, van Venrooij WJ et al. Autoantibodies to cyclic citrullinated peptides predict progression to rheumatoid arthritis in patients with undifferentiated arthritis: a prospective cohort study. Arthritis Rheum 2004; 50: 709–15. 4 Gorman JD. Smoking and rheumatoid arthritis: another reason just to say no. Arthritis Rheum 2006; 54: 10–13.
  13. 13. 6 §01 General Rheumatology and Soft Tissue Rheumatism P R O B L E M 02 An Acutely Swollen/Hot Joint Case History You have been asked to see a 28-year-old man who presents with a 36-hour history of a red and very swollen right knee, upon which he is unable to weight bear. He has been previously well and has no relevant family history. The clinic nurse has recorded his temperature as 37.9°C and a random blood glucose is 7.3 mmol/l. What is your preliminary differential diagnosis? What additional history and examination is relevant? What are the key investigations? How should he be managed? Background Differential diagnosis The knee is one of the most common joints affected by monoarthritis, which is fortunate since it is so easy to aspirate. The differential diagnosis of monoarthritis is listed in Table 2.1. Table 2.1 Differential diagnosis of monoarthritis Trauma – Meniscal or ligamentous tears ± haemarthrosis Sepsis – Gonococcal arthritis, Staphylococcus aureus, penetrating injury, foreign body Reactive arthritis – Following gastrointestinal or genitourinary infection Haemophilia Crystal arthritis – Gout, pseudogout Inflammatory – e.g. Rheumatoid, psoriatic Trauma conjures images of motor vehicle accidents or dramatic tackles in rugby; how- ever, much more mundane twisting injuries or valgus/varus strains when under load are common. A rapidly developing joint swelling within minutes of the injury is suspicious of an anterior cruciate ligament tear with involvement of the blood vessel running along its surface. If internal mechanical derangement is considered possible, then imaging or © Atlas Medical Publishing Ltd
  14. 14. 02 An acutely swollen/hot joint 7 Table 2.2 Common errors in diagnosing acute monarthritis Error Reality The problem is the joint because the patient Surrounding soft tissues, including bursitis, may be the has ‘joint pain’ source of pain The presence of intra-articular crystals excludes Crystals can be present in a septic joint infection Fever distinguishes infectious causes from Fever may be absent in septic monoarthritis, and in the other causes immunocompromised patient. Acute crystal arthritis may cause fever A normal serum urate makes gout unlikely, and a Serum urate is normal for 30% of acute gout attacks, and only high level confirms gout 5% of those with hyperuricaemia develop gout each year Gram staining and culture of synovial fluid are Fastidious, slow-growing organisms, or fragile organisms, may sufficient to exclude infection not be identified in early infection. Liaison with the laboratory is required for specialist media and prolonged incubation orthopaedic review is warranted. Table 2.2 highlights some common errors in diagnosing acute monoarthritis. The presence of fever suggests infection, and the patient should be questioned and examined to determine the likely source. Septic arthritis is usually exquisitely tender with resistance to joint movement. Staphylococci are the most common cause of muscu- loskeletal sepsis, with the prevalence of both streptococcal and mycobacterial infection increasing. For infections with staphylococci, streptococci, Gram-negative bacteria and anaerobes, only one joint is usually involved. Polyarticular involvement is more likely in the elderly or immunosuppressed, with infection by Haemophilus influenza, meningo- cocci and Neisseria gonorrhoeae. Lyme disease can present with knee involvement, although the diagnosis can be quickly excluded if there has been no exposure to the tick vector of Borrelia burgdorferi. In young patients, gonococcal arthritis is the most common non-traumatic acute monoarthritis, and questioning regarding sexual partners and genitourinary symptoms is necessary. In addition to arthritis (often polyarticular), tenosynovitis and a pustular rash of the extremities should be sought. Gonococcal arthritis is 3–4 times more com- mon in women, who often develop arthritis in the perimenstrual period. Men often experience a urethritis as dysuria, and may notice a morning discharge, whereas women may be asymptomatic. Reactive arthritis is a sterile arthritis, occurring distant in both time and place from an inciting infection (usually gastrointestinal or genitourinary). Lower limb asymmetric oligoarthritis is most common, with associated enthesitis such as Achilles tendinitis, and mucocutaneous features of conjunctivitis, pustular rash on the hands and feet and sterile urethritis. Common triggers are genitourinary infection with Chlamydia trachomatis and gastrointestinal infection with Salmonella typhimurium, Shigella flexneri, Campylobacter jejuni and Yersinia enterocolitica. Stool culture and collection of early morning urine for detection of chlamydia DNA by polymerase chain reaction (PCR) should be considered. Crystal arthritis is both dramatic and rapid in onset, with the most commonly impli- cated crystals being uric acid, calcium pyrophosphate and hydroxyapatite. Gout is
  15. 15. 8 §01 General Rheumatology and Soft Tissue Rheumatism unusual in the young and is usually preceded by more distal joint involvement, classically the first metatarsophalangeal joint (podagra). Pseudogout or calcium pyrophosphate dihydrate (CPPD) deposition disease is uncommon below the age of 50 years and the knee is most often involved, followed by wrist and shoulder. Basic calcium phosphate (hydroxyapatite) results in a calcific periarthritis, which most commonly affects the shoulder. Aspirating a knee joint Every medical graduate should feel confident to undertake knee aspiration (Figure 2.1). The knee is exposed with the patient lying so that you can obtain access to either the medial or lateral aspect. The knee is generously cleaned with antiseptic and allowed to dry whilst you are preparing the aspiration syringes. The patella is pinched between thumb and index finger at its midpoint, which allows you to detect tension in the quadriceps muscles and also allows you to distract the patella upwards to increase the infrapatellar space. Local anaesthetic (5–10 ml) is infiltrated via a 21G or 23G needle at a point proxi- mal and inferior to where you are holding the patella, noting that the pain-sensitive structures are the dermis and the thickened synovium as you enter the joint. When the anaesthetic has been given time to work, the joint is aspirated along the same needle track with a fresh 10–20 ml syringe and 18G needle. Afterwards, a dressing is applied firmly for several minutes to ensure haemostasis and to prevent synovial fluid leakage. Figure 2.1 Arthrocentesis of the left knee – medial approach. Only 1–2 ml of fluid is sufficient to complete all investigations; however, the joint should be aspirated of as much fluid as possible without increasing the trauma of the pro- cedure. Substantial pain relief is achieved by aspirating a tense effusion, and while reac- cumulation will occur, it buys some time while the preliminary investigation results are received. As the aspirate is removed, you should note its colour, viscosity and turbidity. Normal synovial fluid is similar to egg white (syn = resembling, ovium = egg) and is both viscous and acellular. As the degree of inflammation increases – from the negligible amount found in osteoarthritis to the mid-range of rheumatoid arthritis and the extreme of septic arthritis – the viscosity decreases and the cellularity and turbidity increase.
  16. 16. 02 An acutely swollen/hot joint 9 Table 2.3 Synovial fluid characteristics Normal Non-inflammatory Inflammatory Septic Colour Clear Straw yellow Yellow Variable Clarity Transparent Transparent Hazy opaque Opaque Viscosity High High Low Low–Thick WBC (¥ 106/l) 0–200 200–2000 2000–75 000 >75 000 Neutrophils <25% <25% 25%–50% >75% WBC, white blood cell. Blood-coloured effusions suggest either trauma or CPPD deposition disease. Synovial fluid characteristics are shown in Table 2.3. It is suggested that approximately 2 ml of fluid is collected into a container plus anti- coagulant, and the remaining fluid collected in a large-volume sterile container. Tests requested should include an urgent Gram stain, cell count and differential count, crystal examination using polarized light microscopy and culture. If gonococcal or fungal infec- tions are suspected, this needs to be highlighted as it influences the culture medium and length of culture required. Analgesics, antipyretics and rest should be employed in the first instance, with the aspiration itself often affording a considerable pain relief. If septic arthritis is suspected, then intravenous antibiotics covering Staphylococcus aureus and N. gonorrhoeae should be commenced after the synovial fluid aspiration. The presence of bacteria on Gram staining or subsequent bacterial growth requires specialist medical and orthopaedic review to combine antibiotic therapy with joint lavage. Gout is confirmed by the presence of intracellular, negatively birefringent urate crys- tals, with intracellular pyrophosphate crystals confirming pseudogout. Both of these con- ditions are self-limited and spontaneously improve over a few days. Adequate hydration combined with analgesia and the introduction of a non-steroidal anti-inflammatory drug will generally suffice. Colchicine at a dose sufficient to impact on acute gout invari- ably causes diarrhoea. If you have confirmed the joint is sterile, then intra-articular corti- costeroid injection provides excellent resolution. Recent Developments 1 A prospective study of children presenting for investigation of possible septic arthritis of the hip concluded that oral temperature >38.5°C was the best predictor, followed by an elevated serum C-reactive protein (CRP), an elevated erythrocyte sedimentation rate, refusal to weight bear and an elevated white cell count.2 CRP >20 mg/l was a strong independent risk factor and a valuable tool for assessing and diagnosing septic arthritis of the hip. As the number of risk factors increases so does the predicted probability of septic arthritis, such that three to five factors present is associated with 83%–98% predictive probability.
  17. 17. 10 §01 General Rheumatology and Soft Tissue Rheumatism 2 Increased plasma procalcitonin (PCT) may be a useful marker for osteomyelitis but not septic arthritis. Procalcitonin is cleaved in neuroendocrine tissues – such as thyroid C cells, lung and pancreatic tissue – to calcitonin. During infection, large amounts of PCT are released. The source is probably monocytes stimulated by endotoxin, and hepatocytes stimulated by tumour necrosis factor or interleukin-6. The role of PCT measurement with a rapid immunoassay was investigated in children admitted with suspected osteomyelitis or septic arthritis.3 The authors reported specificity of 100% and sensitivity of 58% for osteomyelitis and the same specificity, but lower 27% sensitivity, in septic arthritis. 3 High-resolution magnetic resonance imaging (MRI) of soft tissues and joints is increasingly used prior to interventions such as arthroscopy. In a cohort of children, Luhmann and colleagues4 compared radiological interpretation of knee MRI with that of the surgeon who integrated the history, clinical examination, plain radiographs, MRI scans and radiologist report. The pre-operative diagnosis by the surgeon was better (P <0.05) than the formal radiology interpretation with respect to anterior cruciate ligament tear, lateral meniscal tear, osteochondritis dissecans and discoid lateral meniscus. Conclusion An acutely hot, swollen joint is an urgent presentation. Exclusion of sepsis is mandatory, particularly in children and immunocompromised patients. Joint aspiration remains the investigation of choice. Subsequently, treatment will often include antibiotics, pending laboratory results, combined with judicious use of analgesia and anti-inflammatory medications. Analysis of synovial fluid is valuable in establishing the diagnosis of gout, particularly in joints other than the classical podagra of the great toe. Patients often inter- pret the doctor’s ‘it could be gout’ comment about their sore joint as either a definitive diagnosis or as a slur on an indulgent lifestyle, when neither may be intended. Further Reading 1 Siva C, Velazquez C, Mody A, Brasington R. Diagnosing acute monoarthritis in adults: a practical approach for the family physician. Am Fam Physician 2003; 68: 83–90. 2 Caird MS, Flynn JM, Leung YL, Millman JE, D’Italia JG, Dormans JP. Factors distinguishing septic arthritis from transient synovitis of the hip in children. A prospective study. J Bone Joint Surg Am 2006; 88: 1251–7. 3 Butbul-Aviel Y, Koren A, Halevy R, Sakran W. Procalcitonin as a diagnostic aid in osteomyelitis and septic arthritis. Pediatr Emerg Care 2005; 21: 828–32. 4 Luhmann SJ, Schootman M, Gordon JE, Wright RW. Magnetic resonance imaging of the knee in children and adolescents. Its role in clinical decision-making. J Bone Joint Surg Am 2005; 87: 497–502.
  18. 18. 03 Painful shoulders – rotator cuff and frozen shoulder 11 P R O B L E M 03 Painful Shoulders – Rotator Cuff and Frozen Shoulder Case History Mr Lawrence, a 76-year-old retired driver, is having difficulty living independently after returning home following a recent myocardial infarction. On the day of discharge he fell heavily, landing on his left upper arm. His concern is a very painful left shoulder, especially at night and when he tries to move his left arm during the day. How would you determine whether he has adhesive capsulitis (frozen shoulder)? Is there a role for medical imaging, and if so, what modality? What treatment should be initiated? Background Shoulder pain is an almost unavoidable life experience; in one study, 7% of an adult pop- ulation aged 25–75 years reported at least one month’s shoulder pain in the previous year. The peak annual incidence of shoulder disorders is in the fourth and fifth decades, at a rate of 0.25%. A Dutch study found that 25% of all 85-year-olds in Leiden suffered from chronic shoulder pain and restriction. Community-based surveys concur with this high incidence of soft tissue lesions about the shoulder, with roughly equal sex incidence. Up to 20% of patients with chronic symptoms and 65% of all diagnoses relate to lesions of the rotator cuff. Rotator cuff disease is the most common cause of shoulder pain found in these studies. An ultrasound study found rotator cuff tears in 13% of 50–59-year-olds, 20% of 60–69-year-olds, 31% of 70–79-year-olds and 51% of subjects aged over 80 years, even when they were asymptomatic. Table 3.1 summarizes causes of shoulder pain. The pain-sensitive structures of the shoulder are mainly innervated by the fifth cervical segment (C5), so that pain from these structures is referred to the C5 dermatome creating the sensation of pain over the anter- ior arm, especially the deltoid insertion. The acromioclavicular joint is innervated by the C4 segment – pain arising here is felt at the joint itself and radiates over the top of the shoulder into the trapezius muscle and to the side of the neck. Clinical assessment A history of trauma, marked night pain and weakness on resisted abduction strongly sug- gests a rotator cuff tear. The sleeping position that induces night pain is an important clue: © Atlas Medical Publishing Ltd
  19. 19. 12 §01 General Rheumatology and Soft Tissue Rheumatism Table 3.1 Causes and clinical characteristics of shoulder pain Category Cause Clinical features Extracapsular Rotator cuff and subacromial bursa (e.g. impingement Painful arc of abduction lesions syndromes, calcific tendinitis, cuff tears, bursitis) Pain on resisted cuff muscle movements, with intact passive movement (allowing for pain and guarding) Pain on impingement manoeuvres as the inflamed rotator cuff tendons impinge on the inferior surface of the acromion and coracoacromial arch Intracapsular Glenohumeral joint (inflammatory arthritis – RA, Loss of both active and passive movement lesions spondyloarthritis, pseudogout) Reduced glenohumeral range Joint capsule (adhesive capsulitis) Night pain Bone disease (Paget’s disease, metastases) Muscle strength, allowing for pain, is intact Referred pain Cervical spine (facet joint root impingement, discitis) Arm and hand pain with paraesthesia Brachial plexus (brachial amyotrophy) Marked muscle weakness and wasting Thorax (Pancoast’s tumour) Neck pain and stiffness Thoracic outlet syndrome Herpes zoster rash Suprascapular nerve entrapment Systemic features with weight loss Subdiaphragmatic (abscess, blood, hepatic lesions) shoulder pain that results in awakening when not lying on that shoulder is found in adhe- sive capsulitis and inflammatory arthritis; pain when lying on the affected shoulder is seen in acromioclavicular joint disease and rotator cuff disease. Prior shoulder problems sug- gest rotator cuff disease with chronic impingement, or calcific tendinitis. A history of marked shoulder joint swelling suggests inflammatory arthropathy with the presence of an anterior bulge in the shoulder usually secondary to a subacromial bursa effusion. Glenohumeral osteoarthritis (OA) is characterized by morning stiffness, pain with use and chronicity of symptoms. OA, however, is less common than rotator cuff dysfunction. Examination of the shoulder is best undertaken with the patient wearing the mini- mum of upper body clothing. The contours of the shoulder are examined for wasting, asymmetry and muscle fasciculation. Palpation should proceed from the sternoclavicular joint along the clavicle to the acromioclavicular joint, to the tip of the acromion and the humeral head beneath the acromion. The shoulder range of movement should be exam- ined both actively and passively, with muscle strength and pain on resistance assessed. There are essentially three movements to test in the shoulder: abduction due to supraspinatus contraction; external rotation as a result of infraspinatus and teres minor movement; and internal rotation due to subscapularis movement (Box 3.1). Box 3.1 Practice Point Three positive clinical tests (supraspinatus weakness, weakness of external rotation and impingement) or two positive results for a patient older than 60 years are highly pre- dictive of a rotator cuff tear.1 Complete rotator cuff tears will show no active abduction but near full-range movement when passively moved. During examination ask about a painful arc during abduction (Figure 3.1). When examining active and passive abduction you should stand behind the patient and place one hand over the shoulder and scapula. The scapula should not begin to
  20. 20. 03 Painful shoulders – rotator cuff and frozen shoulder 13 Painful arc of abduction acromioclavicular joint 180° Painful arc of abduction in rotator cuff 120° 70° Figure 3.1 Painful arc: the patient slowly abducts the arm as high as possible, describing symptoms as the arm rises. elevate or rotate until at least 90 degrees of abduction has been reached. Early scapulotho- racic movement localizes the abnormality to the glenohumeral joint or capsule, as seen in frozen shoulder. You should examine external rotation at 0 degrees abduction, with the elbow beside the chest, and if external rotation is absent then a frozen shoulder is likely. Next re-examine both internal and external rotation at 90 degrees abduction; if both are restricted, a frozen shoulder is again likely. Bicipital tendinitis is examined by testing resisted flexion at 30 degrees external rotation, and feeling for tenderness in the bicipital groove. Shoulder impingement can be reproduced by internally rotating the arm held flexed at 90 degrees and bringing the inflamed rotator cuff against the anterior acromion. The ‘empty can’ test is suggestive of a rotator cuff tear: it shows pain on resisted elevation of the inverted arm held extended at 90 degrees, as if emptying a can of drink. Rotator cuff disease The glenohumeral joint is, by virtue of its anatomical shape, inherently unstable, relying on the joint capsule as well as the rotator cuff muscles (supraspinatus, infraspinatus and subscapularis) for additional stability. Impingement of the rotator cuff between the prox- imal humerus and the acromioclavicular arch may occur from anomalies of the arch (structural impingement) and from instability due to joint hyperlaxity or weak rotator cuff muscles (functional impingement). Coracoacromial arch anomalies may be congen- ital, dependent on acromial shape. Three shapes have been described – flat, curved and
  21. 21. 14 §01 General Rheumatology and Soft Tissue Rheumatism hooked – although there is poor inter-observer agreement on identifying the shape. Acquired impingement occurs secondary to osteophytes growing from the acromioclav- icular joint or calcification of the acromioclavicular ligament. Impingement occurs when the cuff becomes compressed in the subacromial space as the arm is elevated. As the humeral head rotates, the rotator cuff tendons are compressed between the greater tuberosity of the humerus and the anterior edge of the acromion, the coracoacromial lig- ament, the under-surface of the acromioclavicular joint and with the reactive inflamma- tory subacromial bursa. In addition to the impingement theory, a vascular theory has been proposed. With the arm at the side, it has been suggested that the supraspinatus tendon has a relative avascu- lar area 1 cm proximal to its insertion at the greater tuberosity, directly beneath the impingement zone. This may be affected by the position of the shoulder and increases with age. However, the infraspinatus tendon has a similar vascular watershed area, sug- gesting that factors other than vascularity are important. Chronic irritation in the avascu- lar region produces tendinitis, leading to local inflammation and further compression. Other causes of tendinitis include trauma, instability and possibly infarction of the cuff in patients with vascular disease. The vascular and impingement theories are not mutually exclusive and it is possible that the high incidence of supraspinatus pathology is the result of impingement in and around a critical zone of vascular supply. With time, wearing and attrition of the cuff leads to impaired action or rupture of the short rotators stabilizing the humeral head into the glenoid fossa, so that the deltoid pulls the humerus against the under-surface of the acromion and a vicious impingement cycle is established. Impingement-caused tears are usually incomplete in the supraspinatus and infraspinatus tendons and complete in the long head of biceps. Complications of impingement include a frozen shoulder, rupture of the rotator cuff tendons or long head of biceps and, in elderly patients with a long-standing tear, a feared end-stage lesion, ‘recurrent haemorrhagic shoulder of the elderly’. Treatment depends on the mechanism of impingement. Patients with functional impingement are treated with a resting sling for 24–36 hours, pendular exercises and full- dose non-steroidal anti-inflammatory drug (NSAID). Structural impingement is treated similarly but the surgical options of arthroscopic surgery to remove osteophytes or trim the acromion are available. Corticosteroid injection to the subacromial space can be combined with an initial 4–7 days of pendulum exercises and avoidance of abduction prior to a programme of shoulder-strengthening exercises. Infraspinatus strengthening may be important to provide stabilization of the humeral head to prevent superior sub- luxation on abduction. Studies of eccentric loading exercises have shown promising results, particularly in lesion of the Achilles tendon. Eccentric loading exercises involve a load being applied to a muscle in its contracted position and the muscle is lengthened under the load. In the shoulder, the supraspinatus would be contracted with the arm abducted and under load the arm would slowly return to the side. Exercise programmes require highly motivated people and there is concern that exercises can increase symptoms initially. Frozen shoulder/adhesive capsulitis Initially described in 1872, this condition remains as ‘difficult to treat and difficult to explain from the point of pathology’ as Codman observed in 1934. This common disor-
  22. 22. 03 Painful shoulders – rotator cuff and frozen shoulder 15 der (2% cumulative risk in an at-risk population annually) is frequently misdiagnosed and is characterized by painful restriction of all shoulder movements, both active and passive, with characteristic restriction in the glenohumeral range. There is marked reduc- tion or absence of shoulder external rotation at 0 degrees abduction, reduction of both internal and external rotation at 90 degrees abduction, as well as prominent restriction of placing the hand behind the back on internal rotation. Frozen shoulder is characterized pathologically by fibrosis and retraction affecting predominantly the anterior and infer- ior structures of the glenohumeral joint capsule. Patients usually present in the sixth decade and onset before the age of 40 years is uncommon. Table 3.2 lists the diseases associated with frozen shoulder, diabetes being the most significant. Diabetes, particu- larly long-standing insulin-dependent diabetes, is associated with glycosylation of subcu- taneous collagen and the development of soft tissue contraction – so called diabetic cheiroarthropathy. Table 3.2 Common disorders associated with frozen shoulder • Acute shoulder trauma and shoulder immobilization • Diabetes mellitus • Thyroid disease (both hyper- and hypothyroidism) • Cardiac disease, particularly after cardiac surgery • Neurological disease with loss of consciousness or hemiplegia • Pulmonary disease – tuberculosis and carcinoma • Rotator cuff disease, especially cuff tear • Acute glenohumeral joint inflammation Three phases of frozen shoulder are recognized: 1 Painful inflammatory phase. Beginning insidiously, with often only a minor injury being recalled, nocturnal awakening pain develops. The pain may be constant and prevents the patient lying on the shoulder. Physiotherapy often aggravates symptoms at this stage and corticosteroid injections are of limited benefit. This phase lasts 2–9 months. 2 Frozen shoulder. With time, the night and rest pain eases, but the shoulder remains ‘frozen’. Mean duration is 4–12 months. 3 Recovery phase. After a mean delay of 5–26 months, shoulder limitation slowly recovers in the majority of patients towards normal range (usually a 10%–30% loss of motion, which is often undetected by the patient). The total duration of symptoms lasts 12–42 months, with mean disease duration of 30 months. In 10%–20% of patients a contralateral frozen shoulder develops, usually milder than the first, while the original shoulder is ‘thawing’. It is important to educate patients that the condition will spontaneously resolve and the stiffness will greatly reduce. NSAIDs and analgesics are used but there are no randomized controlled trials studying efficacy. A prospective study in frozen shoulder compared exercise within the limits of pain with intensive physiotherapy. Those who performed exercises within the limits of pain had better results, recorded as near-normal painless shoulder movement (64% of patients at 12 months, 89% at 24 months), compared to intensive physiotherapy (63% of patients at
  23. 23. 16 §01 General Rheumatology and Soft Tissue Rheumatism 24 months).2 An early meta-analysis by Hazleman on the use of intra-articular steroids reported that the outcome depended on the duration of symptoms and hence possible stage of disease. Patients who receive the injection earlier in the course of the disease recover more quickly.3 An extensive meta-analysis by Buchbinder et al. found a benefit for glenohumeral intra-articular corticosteroid injection for frozen shoulder compared with placebo.4 For those unable to tolerate the pain and disability of a frozen shoulder, manipulation under anaesthesia (MUA) is a reliable way to improve the range of movement. It is par- ticularly indicated when disability persists after six months of non-operative therapy. More recently, arthroscopic release of the capsule has been advocated as a more con- trolled release of the capsule than MUA. Arthroscopic release also avoids the complica- tions of MUA such as fracture of the humerus and iatrogenic intra-articular shoulder lesions.5 Imaging Imaging is undertaken primarily when considering referred pain or a malignant process. In the assessment of rotator cuff disease, no imaging may be required initially, and may only be undertaken subsequently if the clinical progression is not as expected. A plain X- ray should then be the initial imaging modality, because if there is marked superior migration of the humeral head, there must be complete rotator cuff disruption. Either magnetic resonance imaging or ultrasound can confirm a possible full-thickness rotator cuff tear, although ultrasound is significantly cheaper and is preferred by patients. Suspected partial-thickness tears are best verified with an ultrasound scan.1 Recent Developments 1 Oral steroids may be useful in frozen shoulder, particularly during the early inflammatory phase. Buchbinder et al.6 undertook a randomized controlled trial on a series of 50 patients and found that oral steroid therapy initially improved the frozen shoulder but the effect did not last beyond six weeks. Their subsequent analysis of five small trials, in which all subjects received physiotherapy or an exercise programme, confirmed that oral prednisolone or cortisone when given for 3–4 weeks had a modest benefit on pain and disability and ability to move the shoulder.7 2 Recently, a neural aetiology for tendinopathy has been considered.8 Tendinopathy was proposed as an appropriate term for a symptomatic primary tendon disorder, as it made no assumption as to the underlying pathological process. Underlying the neural theory are four basic observations: tendons are innervated; substance P has been found in rotator cuff tendinopathy and is a pro-inflammatory mediator; the neurotransmitter glutamate is also present in tendinopathy; and tendon nerve cell endings are closely associated with mast cells. It has been tentatively postulated that neural stimuli secondary to overuse or mechanical irritation lead to mast cell degranulation and release of mediators that begin an inflammatory cascade.
  24. 24. 03 Painful shoulders – rotator cuff and frozen shoulder 17 Conclusion Frozen shoulder is a common and painful condition that impacts adversely on an indi- vidual’s activities of daily living. Despite being self-limited, recovery is protracted and a high proportion of patients do not regain full function. As a condition, it is largely man- aged in the community by primary physicians, physiotherapists and occupational thera- pists. Treatments that aim to mechanically stretch or disrupt the joint capsule (MUA, arthroscopic release or hydrodilation of the capsule) are reserved for those with severe symptoms who have failed to progress with conservative therapy. Further Reading 1 Diehr S, Ison D, Jamieson B, Oh R. Clinical inquiries. What is the best way to diagnose a suspected rotator cuff tear? J Fam Pract 2006; 55: 621–4. 2 Diercks RL, Stevens M. Gentle thawing of the frozen shoulder: a prospective study of supervised neglect versus intensive physical therapy in seventy-seven patients with frozen shoulder syndrome followed up for two years. J Shoulder Elbow Surg 2004; 13: 499–502. 3 Hazleman BD. The painful stiff shoulder. Rheumatol Phys Med 1972: 11: 413–21. 4 Buchbinder R, Green S, Youd JM. Corticosteroid injections for shoulder pain. Cochrane Database Syst Rev 2003; CD004016. 5 Dias R, Cutts S, Massoud S. Frozen shoulder. BMJ 2005; 331: 1453–6. 6 Buchbinder R, Hoving JL, Green S, Hall S, Forbes A, Nash P. Short course prednisolone for adhesive capsulitis (frozen shoulder or stiff painful shoulder): a randomised, double blind, placebo controlled trial. Ann Rheum Dis 2004; 63: 1460–9. 7 Buchbinder R, Green S, Youd JM, Johnston RV. Oral steroids for adhesive capsulitis. Cochrane Database Syst Rev 2006; CD006189. 8 Rees JD, Wilson AM, Wolman RL. Current concepts in the management of tendon disorders. Rheumatology 2006; 45: 508–21.
  25. 25. 18 §01 General Rheumatology and Soft Tissue Rheumatism P R O B L E M 04 Tennis Elbow and Golfer’s Elbow Case History Simon is a 48-year-old labourer. His work has required a large amount of manual screwdriver use, and he presents with a three-month history of an increasingly painful elbow. He now has trouble grasping objects such as a cup. What is the difference between tennis elbow and golfer’s elbow? What are the characteristics of each condition, and what treatment is indicated? Background Tennis elbow Tennis elbow or lateral epicondylitis is an overload injury, which occurs after minor or unrecognized microtrauma to the proximal insertion of the extensor muscles of the fore- arm – particularly extensor carpi radialis brevis. Tennis elbow is the most frequently diagnosed elbow condition (Box 4.1); it occurs commonly in middle life (age 35–55 years) and has an incidence in general practice of 4–7 cases per 1000. Despite its common name, most cases occur in non–tennis players and it is frequently a work-related enthe- sopathy affecting up to 15% of workers in at-risk industries. Operative specimens reveal tendon glycosaminoglycan infiltration and microtears, as well as new bone formation at the attachment site. Both traction injury and ischaemia play a role in its development. Flexion deformity is unusual, occurs late and is minimal. Loss of 20 degrees of exten- sion cannot be attributed to tennis elbow and warrants investigation for arthritis, impingement at the olecranon fossa or a soft tissue mass in the posterior aspect of the elbow. Tennis elbow is usually self-limiting, having an average duration of six months to two years, with 90% of subjects recovering within one year. Various conservative inter- ventions exist including pain-relieving medications, corticosteroid injections, physio- therapy, elbow supports, acupuncture, surgery and shockwave therapy (Box 4.2). Box 4.1 Diagnosis of tennis elbow b Lateral elbow pain with tenderness on palpation just distal to the lateral epicondyle b Worsening pain localizing to the lateral epicondyle on resisted wrist dorsiflexion b X-rays excluding calcific tendinitis, exostoses and osteoarthritis of the radio-ulnar joint © Atlas Medical Publishing Ltd
  26. 26. 04 Tennis elbow and golfer’s elbow 19 Box 4.2 Treatment of tennis elbow b Structured physiotherapy consisting of elbow manipulation and exercise, supple- mented with home exercises and self-manipulation b Practical advice booklet on self-management and ergonomics b Recommend avoidance of corticosteroid injections, as short-term benefit is offset by a poorer longer-term outcome Most important in treatment is activities modification – both frequency and method of performance. In tennis players, common errors are inadequate conditioning, incorrect grip size, faulty backhand style and problems with the racquet and its stringing. In the work setting, a review by an occupational therapist is recommended, particularly focus- ing on pronation/supination movements and grip size. A physiotherapy programme that includes strengthening exercises for the entire upper limb and a graded resistive pro- gramme for wrist dorsiflexors is recommended. In the setting of localized tenderness it is tempting to inject the lesion. As noted in Box 4.2, the short-term gain may be offset by a poorer long-term outcome. The injection technique is a small volume of corticosteroid and local anaesthetic injected into the tendinous insertion of extensor carpi radialis brevis into the lateral epicondyle. As the injection is not into a potential space but into an already tender, dense area, the injection is against resistance and is both uncomfortable and has the risk of steroid tracking super- ficially to the subcutaneous tissues, leading to depigmentation and atrophy. In contrast to other painful overuse syndromes in which total tendon ruptures have been reported (Achilles, biceps, patella), the tendon of the extensor carpi radialis brevis is strongly con- nected and supported by other extensors of the wrist. A small number of patients have recalcitrant lateral epicondylitis and are considered for operative intervention – open, arthroscopic and percutaneous. Operative interven- tions followed for a minimum of two years demonstrate an improvement compared to pre-operative status, with no difference in outcome according to procedure technique.1 Golfer’s elbow Golfer’s elbow or medial epicondylitis is the mirror image of tennis elbow, and is thought also to relate to repetitive traction stress and microtears at the insertion of the forearm flexors (flexor carpi radialis) and pronator teres into the medial epicondyle. It occurs in both professional and amateur sports players, as well as manual workers such as brick- layers. It is much less common than tennis elbow, with approximately one-twentieth the incidence. Similar to tennis elbow, the diagnosis is clinical, with localized tenderness that worsens on resisted wrist flexion and forearm pronation (Box 4.3). Box 4.3 Golfer‘s elbow b Elbow pain at the medial epicondyle b Increasing symptoms on resisted wrist flexion and resisted forearm pronation b Treatment includes modification of activities, upper limb exercises and analgesics
  27. 27. 20 §01 General Rheumatology and Soft Tissue Rheumatism Recent Developments A randomized controlled trial compared the effectiveness of physiotherapy, cortico- steroid injections and a ‘wait and see’ approach in 198 patients with tennis elbow who were randomized to the three treatment arms.2 Physiotherapy was eight sessions of mobilization with movement and exercises plus home exercises and self-manipulation. Injection therapy with triaminolone acetonide (10 mg) and 1% lidocaine was the second study arm. The ‘wait and see’ approach consisted of ergonomic instruction and use of analgesics, heat, cold and braces if needed. At six weeks the main outcome measures (global improvement, pain-free grip strength, assessor’s rating of complaints, severity of elbow pain and elbow disability) were significantly better in the corticosteroid-treated group than in the other groups. However, all groups were improving and the benefit of the steroid injection was short-lived, such that a crossover occurred around twelve weeks, with the one-year results showing physiotherapy superior to corticosteroid injections for all outcome measures. Importantly, at one year, the injection-treated group was signifi- cantly worse on all outcomes compared with the physiotherapy group, and on two out of three measures compared with the ‘wait and see’ group. The corticosteroid injection group also had the most reported recurrences. A similar study with only seven weeks of follow-up confirmed the benefits of steroid injections in the short term.3 Conclusion Tennis elbow is a common problem in general practice and is best treated with the knowledge that it is a self-limiting condition, with the majority of patients improving in the medium term. Whilst corticosteroid injections offer short-term benefit, there is the potential for both short-term adverse effects and the possibility of a worse outcome at one year. Physiotherapy provides benefit that is slower in onset but is more sustained and allows patients to become self-reliant in their own management. Further Reading 1 Szabo SJ, Savoie FH, Field LD, Ramsey JR, Hosemann CD. Tendinosis of the extensor carpi radialis brevis: an evaluation of three methods of operative treatment. J Shoulder Elbow Surg 2006; 15: 721–7. 2 Bisset L, Beller E, Jull G, Brooks P, Darnell R, Vicenzino B. Mobilisation with movement and exercise, corticosteroid injection, or wait and see for tennis elbow: randomised trial. BMJ 2007; 333; 939–45. 3 Tonks JH, Pai SK, Murali SR. Steroid injection therapy is the best conservative treatment for lateral epicondylitis: a prospective randomised controlled trial. Int J Clin Pract 2007; 61: 240–6.
  28. 28. 05 Carpal tunnel syndrome and other entrapment neuropathies 21 P R O B L E M 05 Carpal Tunnel Syndrome and Other Entrapment Neuropathies Case History Beatrix is a 33-year-old production-line worker. For the last four weeks she has been awakening with painful pins and needles in her left hand and a dull pain that radiates from her wrist to her elbow. Shaking the arm improves the symptoms and she sometimes sleeps with her arm hanging out of the bed. What are the clinical features of the carpal tunnel syndrome? What is the role for imaging and nerve conduction studies? What investigations are appropriate to determine the cause? How would you manage this problem? Background Entrapment neuropathies are disorders where peripheral nerves are damaged by com- pression as they pass through a bony or fibrous canal. The disorders may be precipitated by repetitive motion or strain, and carpal tunnel syndrome (CTS) is by far the common- est entrapment neuropathy and the most common focal peripheral neuropathy. The median nerve, along with the flexor tendons, passes through the carpal tunnel, which is bridged by the transverse carpal ligament (Figure 5.1). CTS affects 3% of the population although there is an imperfect correlation between reported symptoms and electrophysi- ological findings. Women are three times more likely than men to be affected with CTS, and a number of predisposing conditions are recognized (Box 5.1). CTS causes pain, numbness and tingling in the distribution of the median nerve: i.e. anteriorly, in the lateral half of the ring finger to the median half of the thumb; and poste- riorly, in the distal halves of the ring and middle fingers. If severe, the symptoms may radiate up the arm and they can often occur at night, thus disturbing sleep. In severe cases there is a loss of small muscle function, which impairs manual dexterity and can lead to wasting of muscles of the thenar eminence. The symptoms of CTS are common and clin- ical signs (Box 5.2) are not always present. Accurate diagnosis is one of the major deter- minants of successful treatment. The diagnosis should be confirmed wherever possible by nerve conduction studies. © Atlas Medical Publishing Ltd
  29. 29. 22 §01 General Rheumatology and Soft Tissue Rheumatism Median nerve Tendon sheath Carpal ligament Bundle of tendons Figure 5.1 Anatomy of the carpal tunnel. Other common nerve entrapment syndromes Thoracic outlet syndromes These are due to compression of the brachial plexus and brachial vessels in the neck. Costoclavicular syndrome, due to a narrowing of the space between the clavicle and first rib, may arise from congenital abnormality or because of poor posture. Cervical rib syn- drome is due either to an extra rib or to a fibrous band between the seventh cervical ver- tebra and the sternum. Compression of nerves and vessels occurs as they pass over the additional structures. Adson’s test may be positive: the patient looks to the affected side and takes a deep breath while the examiner lifts the arm to 90 degrees. If compression is present, the radial pulse may disappear. Suprascapular neuritis The suprascapular nerve (cervical segments C5/C6) supplies sensation to the shoulder joint and motor supply to the infraspinatus and supraspinatus muscles. It can become com- pressed as it passes through the suprascapular notch and under the transverse ligament. Ulnar neuritis Compression of the ulnar nerve usually occurs in the canal, where it is covered by the arcu- ate ligament. It may also occur between the two heads of flexor carpi ulnaris just distal to the elbow joint. The syndrome may occur as a result of direct trauma or fracture, repetitive
  30. 30. 05 Carpal tunnel syndrome and other entrapment neuropathies 23 Box 5.1 Causes of CTS Overuse Repetitive flexion or extension of the wrist, particularly while gripping objects firmly Use of walking stick in patients with mobility disorders Occupational – use of power tools, assembly-line work Injury Colles fracture Subluxation of the lunate bone Arthritis Rheumatoid – tendon sheath inflammation Osteoarthritis Gout or pseudogout Wrist ganglion Outpouching of the wrist joint capsule Increased canal Pregnancy volume Obesity Congestive cardiac failure Lipoma Infections Septic arthritis Lyme disease Tuberculosis Metabolic Diabetes Hypothyroidism Acromegaly Amyloidosis Box 5.2 Clinical signs of CTS Tinel’s sign Tapping over the median nerve elicits symptoms in the distribution of the nerve Phalen’s sign Place both hands together palm to palm, with the wrists extended to 90 degrees, and forearms horizontal and close to the chest. The affected hand will begin to tingle within 1–2 minutes Reverse Phalen’s As above, but with the hands placed back to back movements or rheumatoid arthritis affecting the elbow joint. It causes pain and tingling down the inside of the forearm to the little finger and medial aspect of the ring finger. The nerve gives rise to a sensory supply to the skin of the hypothenar eminence and a motor supply to muscles of the hypothenar eminence and other small muscles in the hand. Median neuritis This is a much less common syndrome and is usually due to entrapment of the nerve at the elbow. Symptoms are similar to those of the carpal tunnel syndrome and may be exacerbated by pronation of the forearm. Radial neuritis Again, this is relatively uncommon. Compression usually occurs at the elbow and causes sensory symptoms in the forearm bone to the base of the thumb.
  31. 31. 24 §01 General Rheumatology and Soft Tissue Rheumatism Meralgia paraesthetica The lateral cutaneous nerve of the thigh passes through the femoral canal, where it is sharply angulated and liable to compression. The syndrome leads to sensory symptoms in the middle and lower part of the lateral aspect of the thigh. It is caused by obesity, direct trauma or by repetitive flexion of the thigh. Anterior compartment syndrome This part of the lower leg contains the tibialis anterior and extensor digitorum muscles and the deep peroneal nerve (supplies skin between the first and second toes). The nerve may be injured by unaccustomed running, as a result of tibial or fibular fractures or through direct trauma. Medial compartment syndrome This is the most common lower-leg nerve entrapment syndrome. The symptoms include pain and tenderness on the medial aspect of the shin (‘shin splints’). It is often precipi- tated by unaccustomed running on a hard surface. Posterior compartment syndrome This compartment contains the soleus and gastrocnemius muscles, which join together to form the Achilles tendon and are responsible for plantar flexion. The syndrome is associated with calf pain precipitated by exercise and with altered sensation on the sole of the foot. The management of all of these nerve entrapment syndromes is somewhat similar: the patient should rest wherever possible and avoid movements or actions that exacerbate the symptoms; local injection with anaesthetic or steroid is indicated in some cases, and a minority of patients require surgical decompression of the affected nerve. Management of CTS1,2 b General measures include trying to relax the grip, using grip-adapted implements such as large pens, taking frequent breaks, keeping the hands warm and considering posture and position (e.g. if using a keyboard, this should be at elbow height). Conservative management with ultrasound has been advocated but there are limited trial data to support this therapy. b Splinting the wrist in neutral position may alleviate symptoms related to soft tissue swelling and is most effective when used soon after the onset of symptoms. Night- time splinting is often sufficient. b Non-steroidal anti-inflammatory drugs are effective in some cases, although improvement may be short-lived. Oral corticosteroids are more effective (e.g. prednisolone 20 mg/day for 2–3 weeks, followed by reducing doses). Diuretics are widely used but are often disappointing in their effect. b The use of local injection of anaesthetic and steroid into the proximal carpal tunnel is supported by trial data. The outcome is probably comparable to that of systemic steroids, but the patient is not exposed to the risk of side effects associated with high- dose steroid therapy. The injection may be directly into the carpal tunnel or proximal to the carpal tunnel. Benefit from local injection may last for up to three months and is increased by concurrent splinting. b For patients who have either severe symptoms or do not respond to conservative measures, surgery is required. This has traditionally been carried out by an open
  32. 32. 05 Carpal tunnel syndrome and other entrapment neuropathies 25 Confirm symptoms are in median nerve distribution History and examination to search for underlying causes Enquire about occupation and repetitive strain Mild symptoms Moderate symptoms with signs Severe symptoms Rest Remove precipitating cause NCS ± imaging Trial of splinting No further action Confirmed diagnosis Conservative measures Local injection Systemic steroids Repeat treatment Consider surgery at 3 months • Open • Endoscopic Figure 5.2 Investigation and management of CTS. Imaging is with high-resolution ultrasound or with MRI. NCS, nerve conduction studies. procedure, which can be performed without admission to hospital. More recently, endoscopic carpal tunnel release through two small incisions has been used by many surgeons. This has the advantage of causing less scarring. Recent Developments 1 Not all patients have ready access to nerve conduction studies. Several studies have shown that high-resolution ultrasound and magnetic resonance imaging (MRI) may be very accurate in diagnosing CTS.3,4 These methods can demonstrate altered
  33. 33. 26 §01 General Rheumatology and Soft Tissue Rheumatism anatomy and decreased volume of the carpal tunnel, and in patients with CTS show the median nerve is swollen distal to the compression. 2 Some familial cases of nerve entrapment are due to inherited anatomical abnormalities. Recently, the condition of hereditary neuropathy with liability to the pressure palsies (HNPP) has been described.5,6 This condition is inherited in an auto- somal dominant manner and is due to a deletion at locus 17p11.2. HNPP is a slowly progressive condition, punctuated by episodes of acute peripheral neuropathy at sites that are liable to nerve entrapment. 3 Endoscopic surgery has revolutionized treatment of CTS. The two-portal endoscopic approach to managing CTS has been adopted in many centres. Although this approach is attractive, recent trials7,8 suggest that it has very little to offer over tradi- tional open surgery. In general, surgical treatment is more successful than medical or conservative treatment in patients with proven CTS.9 Conclusion CTS is the most common form of entrapment neuropathy. Definitive diagnosis is by nerve conduction studies, but ultrasound and MRI are increasingly being used to confirm the diagnosis. It is worth routinely excluding hypothyroidism and diabetes as predispos- ing causes but there is not usually a treatable or identifiable underlying cause. A limited trial of conservative or medical measures is justified in mild cases but surgery is generally required for severe, progressive or unresponsive cases. Further Reading 1 Viera AJ. Management of carpal tunnel syndrome. Am Family Physician 2003; 68: 265–72. 2 Ashworth N. Carpal tunnel syndrome. Clin Evid 2006; 15: 1–18. 3 Wiesler ER, Chloros GD, Cartwright MS, Smith BP, Rushing J, Walker FO. Use of diagnostic ultrasound in carpal tunnel syndrome. J Hand Surg 2006; 31: 726–32. 4 de Noordhout AM. Diagnosing entrapment neuropathies: probes and magnets instead of electrodes and needles? Clin Neurophysiol 2006; 117: 484–5. 5 Sander MD, Abbasi D, Ferguson AL, Steyers CM, Wang K, Morcuende JA. The prevalence of hereditary neuropathy with liability to pressure palsies in patients with multiple surgically treated entrapment neuropathies. J Hand Surg 2005; 30: 1236–41. 6 Koc F, Guzel R, Benlidayi IC, Yerdelen D, Guzel I, Sarca Y. A rare genetic disorder in the differential diagnosis of the entrapment neuropathies: hereditary neuropathy with liability to pressure palsies. J Clin Rheumatol 2006; 12: 78–82. 7 Rab M, Grunbeck M, Beck H et al. Intra-individual comparison between open and 2-portal endoscopic release in clinically matched bilateral carpal tunnel syndrome. J Plast Reconstr Aesthet Surg 2006; 59: 730–6. 8 Atroshi I, Larsson G-U, Ornstein E, Hofer M, Johnsson R, Ranstam J. Outcomes of endoscopic surgery compared with open surgery for carpal tunnel syndrome among employed patients: randomised controlled trial. BMJ 2006; 332: 1473–6. 9 Hui ACF, Wong S, Leung CH et al. A randomized controlled trial of surgery vs steroid injection for carpal tunnel syndrome. Neurology 2005; 64: 2074–8.
  34. 34. 06 Fibromyalgia syndrome 27 P R O B L E M 06 Fibromyalgia Syndrome Case History Sandra is in her early 40s and is seeing you because she hurts from her scalp to her toes. This has been present for at least eight years and is ruining her life. She tires easily and aches with any activity. Her sleeping is restless, she awakes tired and she has an irritable bowel. There are no abnormalities on physical examination. What is fibromyalgia and how would you support this diagnosis? Are there any investigations that might help? What treatment, if any, would you suggest to Sandra? Background Fibromyalgia syndrome (FMS) is a soft tissue musculoskeletal condition with many features in common with chronic fatigue syndrome, the major difference being the pre- dominance of musculoskeletal features in FMS. Diagnosis of FMS is based on the American College of Rheumatology (ACR) criteria (1990): b Pain on both sides of the body b Pain above and below the waist b Pain in an axial distribution b Local tenderness in at least 11 out of 18 defined trigger points (Figure 6.1) The pain is often defined as ‘aching’ or ‘burning’ and varies in intensity and location from day to day. Other features of FMS are shown in Table 6.1. Table 6.1 Frequency of FMS symptoms Symptom % Symptom % Muscular pain 100 Paraesthesiae 52 Fatigue 96 Memory impairment 46 Insomnia 86 Leg cramps 42 Joint pains 72 Poor concentration 41 Headaches 60 Anxiety 32 Restless legs 56 Major depression 20 © Atlas Medical Publishing Ltd
  35. 35. 28 §01 General Rheumatology and Soft Tissue Rheumatism Figure 6.1 Trigger points for the diagnosis of FMS. There are 18 points in total (nine identical locations on each side). Anterior: anterior aspects of C5, C6 and C7; second rib; lateral epicondyle; knee (medial fat pad). Posterior: suboccipital muscle insertions; supraspinatus muscle origin; trapezius (midpoint upper border); gluteal (upper outer quadrants); greater trochanter. Adapted with permission from Borg-Stein 2006.1 Musculoskeletal pain is the most consistent feature of FMS. Fatigue can be almost as debilitating. Disordered sleep is also a very frequent feature and contributes to fatigue and to the mood disturbances. Sleep abnormalities are strongly correlated with the alpha-electroencephalogram (EEG) abnormality and movement disorders including the periodic jerking of arms and legs, teeth grinding (bruxism) and restless legs. Gastro- oesophageal reflux disease occurs with high frequency, as does irritable bowel syndrome. Headaches may be of the migraine or tension type. Facial pain is also relatively common, including discomfort related to temporomandibular joint dysfunction. Psychological and psychiatric morbidity are increased. There is high prevalence of anxiety disorders including obsessive-compulsive disorder and post-traumatic stress disorder.2
  36. 36. 06 Fibromyalgia syndrome 29 Epidemiology and aetiology A number of recent studies2–4 have examined incidence and prevalence of FMS. The esti- mated prevalence is between 1% and 4%. FMS is between two and six times more likely to occur in women. Incidence in the female population has been estimated at 11.3 per 1000 person-years. It can occur at any age but becomes more common with advancing years. FMS has been associated with other rheumatic disorders including rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). There is clearly strong interplay between physical and psychological factors in FMS. The onset of illness may be triggered by physical illness (including viral diseases) or by trauma (including surgery). There is some suggestion that heredity may play a part, with components of the serotonergic and dopaminergic systems being potential candidates for involvement. Some of the symptomatology around the trigger points may be due to increased acetylcholine at the motor endplate causing contraction and shortening of the sarcomere. This may lead to increased energy consumption and increased local blood supply, with resulting local tenderness. A number of local and systemic mediators have been implicated. These include bradykinin, calcitonin gene-related peptide, substance P, tumour necrosis factor-a, interleukin-1, noradrenaline and serotonin. Investigations Routine investigations – including full blood count and biochemistry, plus erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and other inflammatory markers – are within the normal range. Because thyroid disease is common, it is useful to include thyroid function tests. There are no specific endocrine abnormalities. X-ray, computed tomography (CT) and magnetic resonance imaging (MRI) scans are generally normal. There are no specific abnormalities on muscle biopsy, electromyography or nerve con- duction studies. EEG or more formal sleep studies may be requested in patients who have marked sleep disturbance. This may reveal abnormalities including periodic limb move- ment disorder, rapid eye movement (REM) sleep disorder or sleep apnoea. The diagnosis of FMS is one of exclusion and is made clinically. Prognosis, differential diagnosis and treatment The outlook for FMS is variable and the condition tends to become chronic. However, more widespread understanding and clearer definition, along with a more highly devel- oped treatment flow, are beginning to streamline management and improve the outlook. There is a danger that over-enthusiastic investigation might contribute to making the condition more chronic. However, this should not deter the clinician from making a full investigation and the clinical picture warrants it. The major differential diagnosis is other connective tissue disorders, including rheumatoid disease, SLE and scleroderma. Major differential diagnoses of FMS and investigation of the condition are summarized in Figure 6.2. There is no specific treatment for FMS. Therapeutic measures include the following: b General: investigation and clear diagnosis; educating the patient as to the nature of the diagnosis and reassuring them; attention to psychological and social factors, and encouraging the patient to have a normal sleep pattern as well as to engage in physical activity consistent with their state of health and preferences.