When to Suspect Autoimmune or Rheumatic Disease
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Rheumatic diseases are presented with many many manifestations .It is important to be oriented with these manifestations for early diagnosis and treatment
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When to Suspect Autoimmune or Rheumatic Disease
- 1. When to Suspect Autoimmune and Rheumatic Disease Samar Tharwat Radwan Ass. Prof. of Rheumatology & Immunology (Internal Medicine Department ) Musculoskeletal Ultrasound –EULAR Mansoura University
- 4. Hair Hair loss Alopecia Psoriatic rashes (in the hair line)
- 5. CNS History of stroke, weakness, seizure, psychosis: SLE. Mononeuritis multiplex, peripheral neuropathy: vasculitis, SLE Lymph node enlargement in the neck: SLE, lymphoma with sjögren syndrome
- 6. Eyes Dryness: sjögren syndrome. Redness (uveitis): AS Pallor: anemia from many causes in RA or SLE.
- 7. Face Photosensitivity: SLE Red cheeks (butterfly rash): SLE Scaring hyperpigmentation: SLE Parotid gland enlargement: sjögren Telangectasia: scleroderma Dryness: sjögren syndrome Ulcer: SLE (painless), inflammatory bowel disease (IBD), Behcet’s, RA (from methotrexate use)
- 8. Respiratory& Cardiovascular systems SOB, chest pain, palpitation: SLE, RA History of PE/DVT: SLE, antiphospholipid antibody syndrome (APS) History of bronchial asthma: Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg- Straus)
- 9. Gastrointestinal tract History of jaundice: viral hepatitis History of recent gastroenteritis or bloody diarrhea: ReA History of IBD: enteropathic arthritis History of dysphagia: scleroderma History of HBV: vasculitis. History of HCV: chronic HCV can present as RA Ask about risk factors of HBV, HCV and HIV prior to start any disease modifying
- 10. Urinary system Frothy urine: lupus nephritis Hematuria: lupus nephritis, anti- glomerular basement membrane disease(Goodpasture).
- 11. Sexual and obstetric history History of recent STD’s: ReA. History of oral/genital ulcers: Behcet’s disease. History of still birth at any age and/or history of three recurrent abortion: APS
- 12. Lower Limb History of non-palpable purpura, lower limb edema, nodules: vasculitis
- 14. General Exam
- 15. Fever
- 16. A significant proportion of patients with pyrexia of unknown origin (PUO) are found to have a rheumatological cause • Systemic lupus erythematosus (SLE) • Rheumatoid arthritis • Still's disease • Polymyalgia rheumatica • Systemic vasculitis • Crystal arthropathies Gout Pseudogout • Seronegative arthritis Ankylosing spondylitis Psoriatic arthritis Reactive arthritis Enteropathic arthritis Undifferentiated spondyloarthropathy
- 17. Ferritin Currently, measurement of ANA and rheumatoid factor is suggested for all patients. ANCA is measured only later if vasculitis is suspected Examination The history of the course of the fever can give some clues. A double quotidian fever (i.e. twice a day) is typical of Still's disease, whilst morning fevers are seen in PAN.
- 19. 19
- 20. Livedo Reticularis ✗ Mottled, reticulated vascular pattern ✗ Due to alterations in blood flow through the cutaneous microvasculature ✗ AE: physiologic, rheumatic disease ✗ Livedo racemosa 20
- 21. 21 Telangiectasias • Dilatation of the capillaries, arterioles, and/or venules • Erythematous • Blanchable • Anywhere on the skin & mucous membranes • Systemic sclerosis
- 22. Secondary Telangiectasias 22 subacute cutaneous erythematosus Postinflammatory telangiectasia in a patient with dermatomyositis Telangiectatic and avascular patches typical of dermatomyositis Matted telangiectasias of systemic sclerosis Telangiectasias on PIPJ in a patient with systemic sclerosis Typical periungual telangiectasias in this patient with SLE
- 23. Ulceration in Rheumatic Diseases 23 Aphthous ulcer on the lower lip shows a fibrinous base (Behçet’s disease) pyoderma gangrenosum with an undermined ulcer (Behçet’s disease Lupus ulcer Systemic sclerosis ulceration on the digit pulp and the lateral erythema ( Dermatomyositis) Left leg with ulcers in a patient with rheumatoid arthritis Ulcers in vasculitis
- 24. Panniculitis Inflammation of the SC adipose tissue Associations: ✗ infections ✗ oral contraceptives ✗ antibiotics ✗ Sarcoidosis ✗ Behçet’s disease ✗ SLE ✗ Crohn’s disease ✗ primary biliary cirrhosis ✗ idiopathic 24
- 25. Puffy Fingers No clear definition Differential Diagnosis ✗ Systemic Sclerosis ✗ limited cutaneous SSc with ACA ✗ limited cutaneous SSc with Anti-Th/To Antibody ✗ Early SSc with Anti-Topoisomerase I Antibody ✗ Sclerodactyly in dcSSc with Anti-RNA Polymerase Antibody ✗ Mixed connective tissue disease ✗ SSc-Like Disorders ✗ Anti Synthetase Antibody Syndrome ✗ Rheumatoid Arthritis ✗ Overlapping Syndromes 25
- 26. Nodules in Rheumatoid Arthritis 26 • 1 in 4 patients with long-standing RA will have rheumatoid nodules • Seropositive disease • Non-tender, firm , extensor surfaces • Benign nodules: rheumatoid nodules in the absence of RA Rheumatoid nodules
- 27. Psoriasis and Psoriatic Arthritis 27 Psoriatic plaques Guttate psoriasis Inverse psoriasis Sebopsoriasis Erythrodermic psoriasis Pustular psoriasis Psoriatic nail with onycholysis, pitting and discoloration
- 28. Inflammatory Bowel-Associated Spondyloarthropathy 28 Erythema nodosum Pyoderma gangrenosum Pyostomatitis vegetans Cutaneous polyarteritis nodosa Metastatic Crohn’s Sweet’s syndrome
- 29. Reactive Arthritis 29 Erosions on tongue Nail changes include nail dystrophy, subungual debris, and periungual pustules Keratoderma Blennorrhagica
- 30. Systemic Lupus Erythematosus ✗ Acute ✗ Subacute ✗ Chronic Lupus non specific 30 Butterfly rash Oral ulcers Photosensitivity Annular type Psoriasiform Discoid LE lupus panniculitis LE tumidus Raynaud’s phenomenon Livedo reticularis Leukocytoclastic vasculitis
- 31. Neonatal Lupus Erythematosus 31 Periorbital and inguinal distribution pattern of skin lesions of neonatal SCLE in a 2-month- old female baby ( left ). The sternal scar results from the implantation of a cardiac pacemaker for atrioventricular block grade III
- 32. Systemic Sclerosis 32 Skin sclerosis Skin sclerosis (and hyperpigmentation) of trunk Expressionless face Diffuse hyperpigmentation hypopigmentation Salt and pepper pattern Furrowing of the mouth Extensive subcutaneous calcinosis
- 33. Localized Scleroderma ✗ Limited type ✗ Generalized type ✗ Linear type 33 Morphea Guttate morphea Generalized localized scleroderma (3 or more anatomic sites) Disabling pansclerotic morphea Linear localized scleroderma En coup de sabre Parry Romberg syndrome
- 34. Sjögren’s Syndrome 34 Xerosis Angular Cheilitis Erythema annulare Vasculitis Urticarial vasculitis
- 35. Giant Cell Arteritis 35 Scalp necrosis with loss of surrounding hair Ischemic necrosis of tongue Two months after treatment. There has been sloughing of skin of tip of tongue and healing visible temporal artery thickening or nodularityand erythema over the superfi cial temporal arteries
- 36. Kawasaki Disease 36 Macular, morbilliform rash Strawberry tongue Edema of dorsum of both hands Palmar erythema Peeling, exfoliation of hands
- 37. Granulomatosis with Polyangiitis (Wegener’s Granulomatosis) 37 Necrotic purpura Papulonecrotic lesions Livedo reticularis Necrotic lesions Gangrene Oral ulcers Gingivitis
- 38. Henoch-Schönlein Purpura 38 Palpable purpura palpable purpura involving the upper and lower Typical palpable purpura on the buttocks Ecchymoses and purpura of the scrotum; the penis is swollen and ecchymotic Bullous evolution of purpuric lesions
- 39. Behçet’s Disease 39 Oral ulcers Genital ulcers Erythema nodosum Superficial thrombophlebitis Pathergy Reaction
- 40. Parotid gland enlargement Mumps Sjogren’s Syndrome Sarcoidosis IgG4-Related Disease
- 41. Nails (pitting, periangular erythema) Nail pitting Psoriasis Onycholysis Psoriasis Periangular erythema : dermatomyositis
- 42. Specific exam for any joint
- 43. General appearance (Deformity &Swelling) Rheumatoid Arthritis Fusiform swelling Complete subluxation Zig-zag” deformity Piano key styloid Swan neck deformity Boutonnière deformity Z-deformity
- 44. General appearance (Deformity &Swelling) Reactive Arthritis Inflammatory arthritis of the knee Enthesitis at the insertion of the right Achilles tendon MRI that shows enthesitis Dactylitis Sacroiliitis Nonmarginal syndesmophytes Sacroiliitis
- 45. General appearance (Deformity &Swelling) Psoriatic Arthritis Distal interphalangeal joint involvement Toe dactylitis Predominant distal interphalangeal joint involvement Arthritis mutilans Enthesitis. Periostitis
- 46. Inspection Redness (gout ) Redness (septic arthritis ) Sacrs (Prosthetic joint infection ) Rash (Gottron's Papules) Tendons (Tenosynovitis ) Muscles (muscle atrophy )
- 48. Examination of the other systems
- 50. Pulmonary Manifestations There are six CTDs which have significant pulmonary manifestations • Systemic sclerosis (SSc) or scleroderma. • Rheumatoid arthritis (RA). • Systemic lupus erythematosus (SLE). • Sjogren syndrome (SS). • Mixed connective tissue disease (MCTD). • Polymyositis/dermatomyositis (PM/DM).
- 51. Pulmonary Manifestations Pulmonary manifestations include • Airways • Parenchyma • Vasculature • Pleura • Respiratory muscles –– Diaphragm –– Chest wall muscles
- 54. Pleural fluid analysis for rheumatic diseases
- 55. Nervous System
- 56. Nervous System
- 61. Thrombosis
- 62. Close relationship between mechanisms of inflammation and thrombosis in Rheumatic diseases Antiphospholipid (aPL) Antibodies
- 63. Who is at a risk of thrombosis ? Systemic lupus erythematosus Rheumatoid arthritis Vasculitis Behçet’s Disease (BD)
- 64. The Blood
- 65. The Blood in Rheumatology • Autoimmune hemolytic anemia • Red cell aplasia • Microangiopathic hemolytic anemia (MAHA) • Neutropenia and Felty’s syndrome (RA, splenomegaly, and neutropenia) • Leucopenia and neutropenia • Lymphocytopenia • Lymphadenopathy and splenomegaly • Antibodies to clotting factor • Macrophage activation syndrome (MAS)
- 66. Renal System
- 72. • Pericarditis. • Non infective endocarditis • Pulmonary hypertension • Intracardiac thrombosis • Myocarditis
- 73. Low back pain
- 77. Inflammatory markers (ESR and CRP) • The erythrocyte sedimentation rate (ESR), the most commonly used clinical measure of inflammation
- 82. Radiology
- 83. Radiology Gouty arthritis Punched out erosion of the first metatarsophalangeal joint Marked soft tissue selling ,punched out erosion
- 84. Radiology Calcium Pyrophosphate Dehydrate (CPPD) Deposition Disease or Pseudogout Calcification of the articular lining of the knee
- 85. Radiology Rheumatoid Arthritis Erosive changes joint space narrowing, erosions, and diffused osteoporosis