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In the name of God

In the name of God. Scleroderma I. Salehi A., MD., Rheumatologist. Salehi I. Classification of Scl.:. Localized Scleroderma No visceral involvement No Typical hand involvement No Raynaud’s ph. Only sclerosis of one or more skin area Systemic Scleroderma( SSc ). Scleroderma. Salehi I.

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In the name of God

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  1. In the name of God Scleroderma I. Salehi A., MD., Rheumatologist

  2. Salehi I. Classification of Scl.: • Localized Scleroderma • No visceral involvement • No Typical hand involvement • No Raynaud’s ph. • Only sclerosisof one or more skin area • Systemic Scleroderma( SSc ) • Scleroderma

  3. Salehi I. Localized Scleroderma: • Morphea: • Plaque morphea • Guttate morphea • Generalized morphea • Bullous morphea • Deep morphea • Linear Scleroderma • Scleroderma

  4. Salehi I. Plaque morphea: • Local sclerosis in < 2 anatomic sites • Scleroderma

  5. Salehi I. Plaque morphea: • Local sclerosis in < 2 anatomic sites • Scleroderma

  6. Salehi I. Generalized morphea: • Skin sclerosis in > 3 anatomic sites • Scleroderma

  7. Salehi I. Linear Scleroderma: • Children • Growth impairment of the involved extremity • Dermatomal distribution • En coup de sabre • Scleroderma

  8. Salehi I. • Scleroderma

  9. Salehi I. • Scleroderma

  10. Salehi I. • Scleroderma

  11. Salehi I. • Scleroderma

  12. Salehi I. Systemic Sclerosis( SSc ) • Definition: • Multisystem, Chronic, Autoimmune • Overproduction of collagen fibers • Thickened & sclerotic skin • Raynaud’s phenomenon • Visceral organ involvement( fibrosis ) • Scleroderma

  13. Salehi I. Epidemiology: • Annual incidence in USA: 1-2 / 100,000 • Peak onset age: 30-50 y/o • 100 RA, 10 SLE, 1 Scl • F/M ratio: 3/1 • Black > other race • Rare in children • Rare in men < 30 y/o • Scleroderma

  14. Salehi I. Clinical staging of SSc: • Inflammatory stage: • Early, Edematous phase • Arthralgia, Soft tissue swelling • Mistake for RA & other CTD • Indurative stage: • Intermediate fibrotic phase • Hardness & thickness of skin • Typical clinical features • Atrophic stage: • Late phase • Very thin skin • Scleroderma

  15. Salehi I. Clinical classification of SSc: • Limited Cutaneous Scleroderma( LcSSc ) • Diffuse Cutaneous Scleroderma( DcSSc ) • Systemic Sclerosis sine Scleroderma( SsS ) • Overlap syndrome • Pre-Scleroderma • Scleroderma

  16. Salehi I. Limited Cutaneous SclerodermaDistal portion of extremities plusHead & neckAcrofacial • Scleroderma

  17. Salehi I. Diffuse Cutaneous SclerodermaDistal + ProximalHead & NeckTrunkTrunkal • Scleroderma

  18. Salehi I. Intermediate Cutaneous Scl. • Scleroderma

  19. Salehi I. Pathogenesis: • Unknown • Immunological events • Cellular, Humoral • Cytokines • Adhesion molecules • ICAM-1 • VCAM-1 • ELAM-1 • Vascular changes • Raynaud’s ph. • Activated fibrogenic fibroblast • Scleroderma

  20. Salehi I. Classic case of SSc: • A middle age woman with: • Raynaud’s ph • Skin hardness, hyper-hypopigmentation • Masked face, telangiectasia, Micro-ostia • Sclerodactyly, Pitting ulcer • Dysphagia, Pyrosis, Diarrhea • Dyspnea on exersion, dry cough • Arthralgia, Arthritis • Scleroderma

  21. Salehi I. Skin involvement: • Sclerosis & Thickening • The fingers, hand and face are at first • Pruritus and edema in the early stages • The assessment of skin: • Hardness • Tethering • Modified Rodnan skin score: • In 17 distinct areas • Score from 0( nl ) to 3( most severe ) • Scleroderma

  22. Salehi I. • Scleroderma

  23. Salehi I. Skin involvement: • . Face: • Masklike & expressionless • Microstomia( fish mouth ) • Perioral furrowing • Small sharp nose • Decreased skin folds • Matlike telangiectases • Scleroderma

  24. Salehi I. • Raynaud’s phenomenon(RP ): • Pallority 2p • Cyanosis 1p • Redness 1p • Dx. is only by Hx. • Definite RP: repeated episodes of biphasic color changes upon exposure to cold • Possible RP: Uniphasic color changes + numbness or paresthesia • No RP: No color changes upon exposure to cold • Scleroderma

  25. Salehi I. Skin involvement: • Neck sign • Hair loss & Anhidrosis • Generalized hyperpigmentation • Focal hyper/hypo-pigmentation • Sclerodactyly & tapered fingers • Dissolution of terminal phalanges • Scleroderma

  26. Salehi I. Skin involvement: • Periungual telangiectasia • Capillaroscopy: Giant or sausage loop • Pitting ulcer • Calcinosis cutis • Scleroderma

  27. Salehi I. • Scleroderma

  28. Neck sign

  29. Salehi I. • Scleroderma

  30. Salehi I. Organ involvement: • Gastrointestinal • Pulmonary • Renal • Cardiac • Musculoskeletal • Ophthalmic • Others • Scleroderma

  31. Salehi I. GI. Involvement: • 90% involvement, any part • 50% symptomatic • Esophageal: most common • GERD: pyrosis • Dysmotility: dysphagia • Hiatal hernia • Stricture, atrophy • Scleroderma

  32. Salehi I. GI. Involvement: • Watermelon stomach • Wide-mouth diverticula • Pseudo-obstruction • Bacterial overgrowth • Pneumatosis intestinalis • Rectal prolaps, incontinence • Scleroderma

  33. Salehi I. Pulmonary involvement: • 70% involvement • Interstitial Lung Disease( ILD ): • Fibrosing alveolitis • ¾ of lung involvement • Pulmonary Artery Hypertension( PAH ): 10% • Asymptomatic: 1/3 • Exertional dyspnea: most common • Dry cough: common • Chest pain: uncommon • Hemoptysis: Rare • Scleroderma

  34. Salehi I. • Scleroderma

  35. Salehi I. • Scleroderma

  36. Salehi I. Renal involvement: • 50% clinical involvement • 60-80% in autopsy • Sclerodermal Renal Crisis( SRC ): • In 10-20% of DcSSc: ARF with NL U/A • New Pr or RBC in U/A • Abrupt onset of Marked HTN, 10% NL HTN • Pericarditis, CHF • Microangiopathic hemolytic anemia • Activated RAA system + nephrosclerosis • Scleroderma

  37. Salehi I. Renal involvement: • Risk factors of SRC: • Diffuse, advanced skin involvement • Glucocorticoid use • Anti RNA polymerase • Cyclosporine • Screening for SRC: • In high risk pt.: • Daily home BP measurement • In other pt.: • Biweekly BP measurement • In all pt.: • Plasma Cr & Urine Pr Checking • Every 3-6 months • Scleroderma

  38. Salehi I. • Lung Cancer • The risk is increased similarly in both LcSSc & DcSSc • The risk is 5 x nl population • 1/3 of cancer in SSc • Cardiac involvement: • Secondary to HTN or PAH: most common • Pericarditis + effusion • Association between pericardial effusion & ARF • Myocardial fibrosis, Myocarditis • Heart Failure • Angina pectoris • Conductiondisturbances • Arrhythmias • Scleroderma

  39. Salehi I. Musculoskeletal disease: • In early stage of DcSSc: • Edema & swelling of hands • Arthralgia & myalgia • Tendon friction rub • Finger, wrist, elbow, knee, Ankle • Arthritis + RF( - ) • Myositis: overlap • Deforming polyarthritis: RA + Scl • HA arthropathy • OA of DIP & CMC1 • Scleroderma

  40. Salehi I. Neuromuscular involvement: • Cranial, entrapment, peripheral, cutaneous, autonomic neuropathies • Trigeminal neuralgia • CNS: • Headache, Seizures, • Stroke, Vascular disease • Radiculopathy, myelopathy • Myopathy, Myositis, Overlap syndrome • Scleroderma

  41. Salehi I. Genitourinary involvement: • In men: • Erectile dysfunction: 80% • Male impotence • In women: • Sexual dysfunction • Vaginal dryness • Constriction of the vaginal introitus • Dyspareunia: > 1/2 • Scleroderma

  42. Salehi I. • Sjogren’s syndrome • Hypothyroidism • PBC • Difficult pregnancy • Scleroderma

  43. Salehi I. CREST syndrome • Calcinosis • Raynaud’s ph. • Esophageal dysmotility • Sclerodactyly • Telangiectasia • Scleroderma

  44. Salehi I. Lab. Data: • CBC: Anemia; ESR • FANA, 95%, speckled • Scl-70( AntitopoisomeraseI ) • Highly specific for SSc • High risk of ILD • Associated with DcSSc • Anti-centromere antibodies(ACA ) • Associated with LcSSc • AntiPM-Scl: PM + Scl • AntiU3-RNP( Fibrillarin ): SSc+ PAH • Scleroderma

  45. Salehi I. Lab. Data: • Anti RNA polymerase I & III: only in SSc • Anti RNA polymerase II: in SLE or SSc • RF: 25% • BUN / Cr, U/A • X-Rays: CXR, . . . • Manometry, Spirometry, DLco, • Skin biopsy • Scleroderma

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