7 13 Connective Tissue Disease

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7 13 Connective Tissue Disease

  1. 1. Connective Tissue Diseases Sjogren’s Syndrome Idiopathic Inflammatory Myopathies IIM, Scleroderma
  2. 2. • A 52-year old female grade-school teacher presents with a two-year history of extreme fatigue, and oral dryness. She has noticed increased difficulty getting through a full day at school due to muscle/joint pain, fatigue, and difficulty speaking. She is menopausal. You ask about dryness of the eyes and she admits to frequent use (4 times per day) of artificial tears and photosensitivity. She describes some type of rash when she goes into the sun.
  3. 3. • The oral exam is significant for mucosal dessication, fissured tongue, and dental decay. She exhibits muscle trigger point tenderness on the back of the neck, shoulder, elbows, knees, lower back and hips. She complains of joint pain in the knees and hands. No signs of vasculitis, skin rash, or joint swelling are observed.
  4. 4. Which of the following should be included in the differential diagnosis? ? • Fibromyalgia • Primary Sjogren’s syndrome • SLE • Secondary SS with SLE • Secondary SS with RA • All of the above
  5. 5. Sjogrens syndrome • A chronic, systemic, autoimmune, inflammatory disorder with lymphocytic infiltration , destruction of exocrine glands (lacrimal , salivary) and production of autoantibodies. • Xerostomia -dry mouth • Keratoconjunctivitis sicca- dry eyes • Also affects: lungs(pneumonitis), kidneys ( instertitial nephritis), heart, skin (vasculitis), nervous system, hematopoietic system.
  6. 6. Epidemiology • Incidence 4/ 100 000 • F / M – 9:1 • Onset 35- 50 years of age • Genetic predisposition • Family clustering
  7. 7. SS • Primary SS – K.Sicca – aqueous tear deficiency – Xerostomia – hyposalivation • Secondary SS – Primary SS + another Rheumatological condition • RA, SLE, Ssc
  8. 8. Clinical course SS • Slowly progressive • Risk of developing Lymphoma 3-5% in life time in Primary SS • Mortality ratio for PSS similar to normal population
  9. 9. Systemic involvement • Cutaneous – dryness and pruritus • Vasculitis – – palpable or non palpable purpura of the lower extremities – In crops and may ulcerate – Urticarial vasculitis – Nodular vasculitis
  10. 10. Systemic involvement • Upper airway ds – Recurrent non-allergic rhinitis / sinusitis – Dry cough – Bronchial dryness – mucus plugging • Lung Disease – ILD – most common – Bibasilar crepitation, before cough/DOE
  11. 11. Systemic involvement • Heart Ds • Echocardiographic evidence of past pericarditis • Hypokinesia LV • Congenital heart block in infants and adults related to anti – SSA
  12. 12. Systemic involvement • GI and Hepatic – Disphagia – Esophageal dysmotility – Nausea, epigastric pain, dyspepsia – Primary biliary cirrhosis
  13. 13. Systemic involvement • Renal Disease – Instertitial nephritis • Dysuria • Urinary frequency • Nocturia • And urgency in the absence of infection – Mild proteinuria
  14. 14. Systemic involvement • Genitourinary – Dyspareunia – Interstitial cystitis • Neuropathies – Peripheral – “glove and stocking” • Fatigue • Endocrine disorders – thyroid ds
  15. 15. Other clinical features • Non – erosive arthritis/arthralgias • Raynaud’s • Lymphadenopathy • Vasculitis • Myositis
  16. 16. Criteria for the classification of Sjögren’s syndrome • Ocular Symptoms • Oral Symptoms • Ocular Signs • Schirmer test < 5 mm • Rose Bengal score ³ 4 • Histopathology ³ 1 agglomeration of 50 or more mononuclear cells/4mm • tissue (focus score) • Objective evidence of salivary gland involvement • Autoantibodies • SSA/Ro, SSB/La, ANA, RF • (4 or > high sensitivity and specificity) • Exclusions: lymphoma, sarcoid, GVH, acquired immune deficiency
  17. 17. Sjögren’s syndrome: evaluation • Schirmer test – measures tear production via mm of wetness • Rose Bengal or fluorescein stain – detects disruption or devitalized tissue • Salivary flow – amount of saliva produced • Dental evaluation • Minor salivary gland (lip) biopsy – looking for lymphoid infiltration • Serologic tests (SSA(Ro), SSB(La), ANA, RF) • SPEP, cryoglobulins • Lymph node biopsy • Evaluation for renal tubular acidosis
  18. 18. SS – diff dx oral dryness • Drugs • Tricyclics antidepressants • Cold remedies • Antihystamines • Diuretics antihypertensives • Anti – cholinergics • Acute anxiety or depression • Mouth breathing • Autoimmune Ds • Viral cond. HIV, Hep C • Central brain lesions – Alzheimer’s, Multiple Sclerosis • Head and Neck radiation • Congenital absence of salivary glands
  19. 19. Questions-hyposalivation • Do you have to drink water at night? • Do you keep water at bedside? • Can you swallow a cracker w/o water? • Do you become easily choked? • Recent increased dental decay? • Change in the way food taste ? • Yes response – support hyposalivation
  20. 20. DDx for SS • SLE • Sarcoidosis • Fibromyalgia • Fatigue and depression • Other causes for parotid gland enlargement
  21. 21. Diagnostic procedures • H&P • Lab studies – CBC, BUN, Creatinine, LFT’s, Hep C, HIV, RF, ANA, anti Ro/SSA, anti La/SSB – Sm RNP – Immunoglobulins, UA • Chest Xray • Rose bengal, Schirmer test, Salivary flow rate by Tech 99 uptake, MRI • Labial salivary gland biopsy
  22. 22. Treatment for SS • First Goal symptomatic • Minimize serious outcomes • Lung transplant • Blindness • Loss of teeth • Depression and • Disability
  23. 23. Pharmacological Management • Ocular dryness • OTC eye drops – Systane and Refersh tears • Rx: Cyclosporine eye emulsion 0.05% (Restasis)
  24. 24. Pharmacological Management • Oral Dryness – OTC: Sugar free chewing gum, and lozenges – Rx: • Evoxac (Cevimeline) TID • Salagen (Pilocaroine) 3-4 time per day (Both muscarinic cholinergic agonists) – Avoid in patients with angina, heart block, glaucoma, severe asthma – Side effects: sweating, flushing , polyuria, visual blurring and decreased nigh vision
  25. 25. Oral health • To treat Xerostomia, Candidiasis, dental and periodontal problems • Oral hygiene, tooth brusing, , flossing, dental prophylaxis every 4 months, fluoride applications • Artificial saliva • Avoid drugs that worsen dry mouth
  26. 26. Oral Candidiasis Treatment • Mycelex (Clotrimazole 10 mg lozenges) • suck on one 4-5 times per day • Fluconazole 100mg – Two tables PO on day one , then one per day for two weeks
  27. 27. Systemic therapies • Plaquenil (Hydroxychloroquine) • Arthralgias, skin manif. and fatigue • Methotrexate • Prednisone • Rituximab
  28. 28. Sjögren’s syndrome: parotid gland
  29. 29. Sjögren’s syndrome: B-cell lymphoma, parotid gland (clinical and photomicrograph )
  30. 30. Sjögren’s syndrome: cornea (Rose Bengal stain)
  31. 31. Sjögren’s syndrome: Schirmer test
  32. 32. Sjögren’s syndrome: xerostomia
  33. 33. Sjögren’s syndrome: parotid gland (photomicrograph)
  34. 34. Sjögren’s syndrome: parotid gland (sialograms)
  35. 35. • CASE # 2 • 57 year old white female presents to the primary care clinic to establish care. She has noticed thickening of the skin on her hands that she initially attributed to an allergy to dishwashing detergent. However, she has become increasingly concerned since it has traveled up to her elbows bilaterally and started involving her feet and shins. She has difficulty making a fist due to the skin thickening.
  36. 36. • Additionally, she has been noticing weakness doing simple tasks like getting up off a chair. Physical exam reveals sclerodactyly extending up to the elbows on the upper extremity and up to the mid-shins on the lower extremity. There is evidence of muscle wasting over the biceps and quadriceps with 3/5 muscle strength of the hip flexors. Initial laboratory evaluation was unremarkable except for an elevated CPK of 10,000.
  37. 37. What is the diagnosis? • Limited Scleroderma • Diffuse Scleroderma • Scleroderma/myopathy Overlap • Polymyositis
  38. 38. • The patient in this case presents with skin involvement suggestive of limited scleroderma. However, the proximal muscle weakness with elevated CPK is suggestive of muscle inflammation or myopathy. Muscle encasement due to sclerodermatous involvement of the fascia can cause elevated CPK and weakness as can a concomitant inflammation of the muscle itself. There is no data in the case to make the definitive diagnosis of polymyositis such as a muscle biopsy or antibodies therefore the answer is:
  39. 39. • C, scleroderma with some form of myopathy. While this disease conglomerate can be seen in conjunction with several other autoimmune diseases including SLE, nothing in this patient presentation seems to suggest a diagnosis of SLE. Classical skin manifestations of SLE are malar/discoid facial rash, alopecia and oral/nasal ulcers, which this patient does not have.
  40. 40. Subsets of Systemic Sclerosis • Diffuse cutaneous syst.sclerosis • Limited cutaneous syst.sclerosis • Overlap syndromes • Diffuse or limited with features of other CTD • Mixed connective tissue disease MCTD • Localized sclerodrema • Morphea • Linear scleroderma
  41. 41. Scleroderma • Systemic sclerosis – With diffuse scleroderma: rapidly progressive skin thickening (proximal to elbows and knees), early visceral disease (lung, heart and kidney) – With limited scleroderma: restricted and non progressive skin thickening (distal extremities), delay visceral involvement (CREST) – With overlap: diffuse or limited with features of other CTD (PM, DM, SLE)
  42. 42. SSc Pathogenesis • Susceptible host • Triggering event • Activation immune system • Endothelial cell activation • Activation fibroblasts • Obliterative vasculopathy and Fibrosis (increased collagen deposition)
  43. 43. SSc Epidemiology • Incidence 15-20 cases per million • Females predominant F:M – 5:1 • Age of onset 30-50 years of age • More severe in African American
  44. 44. Scleroderma • DCSS- – proximal and distal skin thickening involves face / neck and trunk – Symmetric involvement fingers, hands, arms and legs – Rapid onset after Raynauds – Auto-Ab present – Overall prognosis poor
  45. 45. Scleroderma • LCSS: – CREST – Limited to symmetrical changes on fingers(sclerodactyly), distal arms and face and neck – Later visceral disease – Abs present – Good prognosis
  46. 46. Scleroderma Cutaneous Manifest. • Skin thickening • Telangectasias • Digital pitting scars • Calcium deposition • Skin ulceration
  47. 47. Other clinical manifestations • Musculoskeletal – Arthralgias and myalgias – Synovitis, tendonitis • GI – Small oral aperture – Esophageal dysfunction – Bowel dysmotility
  48. 48. Cont. • Pulmonary: – Fibrosis and inflammation with ILD • Cardiac – Myocarditis, pulmonary HTN, arrhythmias • Renal – Scleroderma renal crisis, renal failure
  49. 49. ACR systemic sclerosis: preliminary classification criteria • Major criterion or • two minor criteria for diagnosis • Major criterion • Proximal scleroderma • Minor criteria • Sclerodactyly • Digital pitting or scars or • loss of substance from finger pad • Bibasilar pulmonary fibrosis
  50. 50. Scleroderma-like syndromes • Toxin- or drug-induced scleroderma – Organic solvents and epoxy resins – Eosinophilic myalgia syndrome (L-tryptophan) – Bleomycin • Vibration injury • Scleromyxedema • Eosinophilic fasciitis • Graft-versus-host disease
  51. 51. Raynaud’s phenomenon • Episodic, reversible digital skin color change – white to blue to red – well-demarcated • Due to vasospasm • Usually cold-induced • Primary (Raynaud’s disease) and secondary forms
  52. 52. Causes of secondary Raynaud’s phenomenon • Connective tissue diseases – Scleroderma, systemic lupus erythematosus, MCTD, undifferentiated CTD, Sjogren’s syndrome, dermatomyositis • Occlusive arterial disease – Atherosclerosis, anti-phospholipid antibody syndrome, Buerger’s disease • Vascular injury – Frostbite, vibratory trauma • Drugs and toxins – Beta blockers, vinyl chloride, bleomycin, ergot, amphetamines, cocaine • Hyperviscosity/cold-reacting proteins – Paraproteinemia, polycythemia, cryoglobulinemia, cryofibrinogenemia, cold agglutinins
  53. 53. Raynaud’s phenomenon: hands
  54. 54. Scleroderma: Raynaud’s phenomenon, cyanosis of the hands
  55. 55. Scleroderma: skin induration, hands
  56. 56. Scleroderma: acrosclerosis
  57. 57. Scleroderma: acrosclerosis and terminal digit resorption
  58. 58. CREST syndrome: calcinosis cutis, fingers
  59. 59. Scleroderma: calcinosis, hands
  60. 60. Scleroderma: leg ulcer
  61. 61. Scleroderma: facial changes, lateral view
  62. 62. Scleroderma: Mauskopf, facial changes
  63. 63. Scleroderma: Mauskopf, facial changes
  64. 64. Linear scleroderma: en coup de sabre, scalp and forehead
  65. 65. Linear scleroderma: thigh and leg
  66. 66. Morphea: leg
  67. 67. Scleroderma: Raynaud’s phenomenon, hand (arteriogram)
  68. 68. Raynaud’s phenomenon: hand (angiogram)
  69. 69. Scleroderma: acrolysis (radiographs)
  70. 70. Scleroderma: calcinosis and acrolysis (radiograph)
  71. 71. CREST syndrome: arm (radiograph)
  72. 72. Scleroderma: pulmonary fibrosis (radiograph)
  73. 73. Scleroderma: wide-mouthed diverticula, colon (radiograph)
  74. 74. Scleroderma: kidney (arteriograms)
  75. 75. Scleroderma • Rare connective tissue disease that has – Fibrosis – Vascular instability (intimal proliferation and Raynaud’s) – Autoimmunity
  76. 76. Is it autoimmune? • In some patients with scleroderma the ANA is positive • Greater than 80% in limited scleroderma and 50% in diffuse scleroderma
  77. 77. CREST • Old fashioned term but still used – Calcinosis – Raynaud’s – Esophageal dysmotility – Sclerodactyly – Telangiectasia
  78. 78. Limited vs Diffuse Scleroderma Limited Diffuse • Most positive ANA • 50% ANA positive • 80% anticentromere usually nucleolar • Rare renal, heart, lung • Scl 70 in 30%, involvement correlates with • May develop PAH in pulmonary fibrosis long standing disease • Renal crisis with RNA polymerase • Higher mortality
  79. 79. Sclerodactyly and pigmentation
  80. 80. Raynaud’s • Primary – Not associated with any other disease Secondary Associated with connective tissue diseases such as SLE, scleroderma, RA, Sjogren’s, Polymyositis Reversible color change of the digitals with pallor and then rubor and or cyanosis
  81. 81. Treatment: Raynaud’s • Calcium channel blockers • Cold avoidance • Smoking cessation • Other drugs
  82. 82. Proof of Treatment in Raynaud’s associated with Scleroderma • Calcium channel blockers, esp Nifedipine – Small trials, can’t prove effectiveness for healing of digital ulcers Meta-analysis Arthritis Rheum 2001 44:1841-7
  83. 83. Prostacyclins/ Prostaglandin analogues • Iloprost • Very effective in IV(5 trials), less effective po (1 trial) • Effective in RP frequency and severity of attacks and at healing and preventing digital ulcers • Beraprost - effective for recurrent digital ulcers J Rheumatol 1999, 26:2173-8
  84. 84. Treatment: GI Tract • Proton pump inhibitors are very effective for GERD • Anti-reflux maneuvers include: not eating after supper, raising the head of the bed, pro-kinetic drugs to propel food through the stomach • Small bowel overgrowth can be treated by antibiotics on an intermittent basis • Some drugs can help the bowels contract
  85. 85. Incontinence can also occur secondary to: • Hypotonic bowel • Poor anal sphincter tone Diverticulosis can also occur in the bowel
  86. 86. Renal Involvement • Renal crisis is a condition with high blood pressure (usually), hemolysis (intra- vascular), and worsening renal function • Renal crisis is secondary to poor blood flow to the kidney, as well as kidney changes with scarring around the blood vessels
  87. 87. Renal Involvement (cont’d) • Treatment has improved the mortality from scleroderma renal crisis, particularly rapid control of the blood pressure using ACE inhibitors • Some patients do go on to temporary or permanent dialysis
  88. 88. Lung Involvement Two main types: • Interstitial lung disease (inflammation and scarring of the lung parenchyma) • Pulmonary hypertension with high pressures in the arteries perfusing the lungs
  89. 89. Prevalence in Scleroderma Related PAH • Pulmonary Hypertension (PAH) in scleroderma is either – Primary (vascular defect) – Secondary (Secondary to pulmonary fibrosis) – Or both
  90. 90. Treatment of PAH • Same as that for Primary Pulmonary HTN (PPH) Vasodilators such as calcium channel blockers Endothelin receptor antagonist (Bosentan) Prostacyclin analogs: Epoprostenol (Flolan), Iloprost Anticoagulation Treatment of CHF and dysrythmia Oxygen Nitric Oxide
  91. 91. Bosentan • Bosentan (Tracleer) is an Endothelin-1 antagonist – Endothelin-1 is a potent vasoconstrictor and smooth muscle mitogen
  92. 92. Heart Involvement • Patients with scleroderma can develop a cardiomyopathy with thickening heart muscles and reduced blood flow to the heart • This is manifest by shortness of breath, angina or congestive heart failure • It is treated the same way as congestive heart failure from other causes
  93. 93. Pleural and Pericardial Effusions • These can occur in scleroderma, particularly in those with diffuse scleroderma • Sometimes treated with prednisone
  94. 94. Arthritis in Scleroderma • Many patients with scleroderma have arthralgia • Some have inflammatory arthritis with swollen joints • 20% on x-ray can have joint destruction • This is treated with anti-inflammatories, physiotherapy, and sometimes disease- modifying drugs
  95. 95. Calcinosis • Calcium deposits are under the skin in pressure areas • They can break open and ooze white, chalky material • They are often painful
  96. 96. Digital Tuft Resorption • Patients with scleroderma can lose mass at their fingertips making them painful and appearing tapered or shortened
  97. 97. Digital Ulcers • Digital ulcers occur in many patients with scleroderma • They are painful • May take a long time to heal sometimes resulting in gangrene or rarely necessitating amputation • Treatment with analgesics, blood thinners, and new drugs (ie. Bosentan) are being studied for increased healing and a decrease in new ulcers
  98. 98. Digital Ulcers
  99. 99. other treatments are underway, including: • Biologic drugs, such as antibodies to decrease TGF-beta (tumor growth factor beta), which is important in causing fibrosis in scleroderma • Other biologic trials are being considered for patients, such as blocking cTGF, this is important in fibrosis and fibroblast production in scleroderma, and is also an important target
  100. 100. Targeted Therapies • Anti TGFbeta antibodies – under development, 1st trial negative • cTGF antibodies • Targeting pathological pathways • Stem cell transplant study
  101. 101. Scleroderma Mortality • Similar to breast cancer (50% 5 year survival) • From: – Interstitial lung disease – Cardiomyopathy – Pulmonary Hypertension – “We have found that those with renal involvement still have a very high mortality and was the highest association of mortality in our cohort”
  102. 102. Conclusions • Scleroderma is a rare connective tissue disease • It is accompanied by a lot of morbidity and at times mortality • There are good treatments for symptom control of various organ systems • There are some good treatments for reversing the progression of the organ-specific disease, such as scleroderma renal crisis • The future appears promising for direct targets that may help in the treatment of scleroderma
  103. 103. Idiopathic Inflammatory Myopathies IIM
  104. 104. Idiopathic Inflammatory Myopathies (IIM) • Adult polymyositis (PM) • Adult Dermatomyositis (DM) • Juvenile myositis (JDMS) • Malignancy-associated myositis • Myositis overlap with another rheumatic disease • Inclusion body myositis (IBM)
  105. 105. IIM • heterogeneous group of autoimmune disorders characterized by muscle weakness, inflammation and possible systemic complications.
  106. 106. IIM Epidemiology • Rare disease • Incidence 5-10 cases/ million • Prevalence 50-90 cases /million • F:M 2-3: 1 • African American women more affected
  107. 107. IMM Clinical • Proximal and symmetric muscle weakness • Functional deficit from weakness • Difficulty raising their arms, combing the hair, getting up from the chair, walking up steps, frequent falls • Fatigue, joint pain , anorexia • Elevated muscle enzymes: • CK, AST, ALT, aldolase, and LDH • EMG abnormal • Abnormal muscle biopsy
  108. 108. Proposed diagnostic criteria for polymyositis and dermatomyositis • PM diagnosed as definite with 4 out of 5 of the below criteria or probable with 3 out of 5 • DM diagnosed as definite with rash plus 3 out of 4 of the below criteria or probable with rash plus 2 out of 4 criteria – Symmetric proximal muscle weakness – Elevated muscle enzymes (CPK, aldolase, transaminases, LDH) – Myopathic EMG abnormalities – Typical changes on muscle biopsy – Typical rash of dermatomyositis
  109. 109. Polymyositis: differential diagnosis • Polymyositis and dermatomyositis • Hypothyroidism • Drug-induced myopathies • Corticosteroids, colchicine, HMG-CoA reductase inhibitors, zidovudine, hydroxychloroquine, alcohol • Infections – Viral, toxoplasmosis, trichinosis, bacterial pyomyositis • Connective tissue disorders – Lupus, scleroderma, MCTD • Systemic vasculitis – PAN, Wegener’s granulomatosis
  110. 110. Polymyositis: differential diagnosis, cont’d • Metabolic myopathies – Disorders of carbohydrate and lipid metabolism • Electrolyte disturbances – Hypernatremia, hyponatremia, hypokalemia, hypophosphatemia, – hypocalcemia • Inclusion body myositis • Sarcoid myopathy • Amyloid myopathy • Neurologic disorders – Myasthenia gravis, motor neuron disease, muscular dystrophy •
  111. 111. Inclusion body myositis • Males affected more than females • Age of onset usually greater than 50 • Slowly progressive • Distal and asymmetric muscle weakness • Myopathic and neuropathic changes on EMG • Mononuclear cell infiltrates and vacuoles containing amyloid on • muscle biopsy • Responds poorly to corticosteroids
  112. 112. Myositis-specific antibodies ANTIBODY DISEASE ASSOCIATION PREVALENCE Anti-tRNA Dermatomyositis, 20% synthetases (Jo-1) interstitial lung disease, “mechanic’s hands” Anti-SRP (signal African-American women, Rare recognition protein) poor prognosis Anti-Mi-2 Older women, “shawl 5% sign,” good prognosis PM/SCL Polymyositis/scleroderma Rare overlap
  113. 113. Dermatomyositis: heliotrope rash
  114. 114. Dermatomyositis: diffuse facial erythema
  115. 115. Dermatomyositis: rash, chest
  116. 116. Dermayomyositis: macular rash and acanthosis nigricans
  117. 117. Dermatomyositis: “mechanic’s hands”
  118. 118. Dermatomyosistis: periungual involvement
  119. 119. Dermatomyositis: nailbed
  120. 120. Dermatomyositis and scleroderma: periungual involvement (nailfold capillaroscopy)
  121. 121. Dermatomyositis: rash, knees
  122. 122. Dermatomyositis: subcutaneous calcification, knees
  123. 123. Steroid myopathy: muscle (photomicrographs)
  124. 124. Inflammatory myopathy (photomicrograph)
  125. 125. Polymyositis: heart (photomicrograph)
  126. 126. Dermatomyositis: calcinosis, thigh (radiograph)
  127. 127. IIM • Dermatomyositis • Polymyositis • Peaks in kids and • Any age older adults • No rash or • In elderly may be photosensitivity perineoplastic – • Not perineoplastic adenoca usually usually • Rash, photosensitivity • Worse if interstitial lung disease (anti Jo1)
  128. 128. IIM • Rare • Diagnosis made by esp proximal muscle weakness, elevated CK • Muscle biopsy shows degeneration and regeneration of m bundles or with dermatomyositis perivascular inflammation • EMG – spontaneous fibrillation potentials, abnormal action potentials
  129. 129. Other features • Heliotrope rash • Gottren’s sign/papules • Photosensitivity • Mechanics hand • Livedo reticularis • Some are overlaps with other connective tissues diseases
  130. 130. Antibody and Lab profile • Increased CK (or aldolase), normal CBC • Occ increased ESR • May have + ANA, ENA such as PM/Scl or Jo1 • Jo1 correlates with interstial lung disease and has a bad prognosis and is very specifici • PM/Scl- often with scleroderma polymyositis overlap
  131. 131. Treatment • High doses of steroids • Steroid sparing drugs such as Imuran, Methotrexate, Cytoxan • Treatment and prevention of complications such as steroid induced osteoporosis • Biologics possibly (TNF inhibitors) • Lung disease needs aggressive treatment
  132. 132. Complications • Esophageal involvement – aspiration • Cardiac involvment – arrhythmia • Cancer associated – death • Heterotopic muscular calcification • Raynauds – ulcers • Sclerodactyly – flexion contractures

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