CLINICAL MANIFESTATION OF SYSTEMIC SCLEROSIS Ahad Azami M.D. Rheumatologist

Clinical Features of SLE OUTLINE: Definition Epidemiology Clinical Features of SLE

Systemic Sclerosis (scleroderma) Systemic sclerosis is a chronic multisystem disorder of unknown etiology characterized by the overproduction and accumulation of collagen with thickening of the skin and by structural and functional abnormalities of visceral organs, including the GI tract, lung, heart and kidneys

CLASSIFICATION A.SYSTEMIC SCLEROSIS 1.Diffuse cutaneous scleroderma 2.limited cutanous scleroderma (CREST syndrome) 3.Sestemic sclerosis sine scleroderma B.LOCALIZED SCLERODERMA 1.Linear 2.Morphea

EPIDEMIOLOGY PREVALENCE:19-75 / 100000 FEMALE / MALE:3 / 1 AGE:30-50

CLINICAL MANIFESTATION

Systemic Sclerosis Diffuse Limited Skin Involvement Distal and proximal extremities, face, trunk Distal to elbows, face Raynaud’s Phenomenon Onset within one year or at time of skin changes May precede skin disease by years Organ Involvement Pulmonary (interstitial fibrosis); renal (renal crisis); gastrointestinal; cardiac GI, pulmonary hypertension Nail Fold Capillaries Dilation and dropout Dilation without significant dropout Antinuclear Antibodies Anti-topoisomerase Anti-centromere

Clinical Features of Systemic Sclerosis (% Patients during course of disease) Limited Diffuse Raynaud’s 95-100 90-95 Skin thickening 98 100 Subcutaneous calcinosis 50 10 Telangiectasia 85 40 Arthralgias/arthritis 70 Myopathy 5 Esophageal dysmotility 80 Pulmonary fibrosis 35 Pulmonary HTN 1 CHF 30 Renal crisis 15

RAYNOUD’S PHENOMENON

SKIN CHANGES 1.Edematous phase 2.Indurative phase(Sclerotic phase) 3.Atrophic phase

EDEMATOUS PHASE

INDURATIVE PHASE

SCLEROTIC PHASE

CALCIFICATION

PITTING SCAR

CAPILAROSCOPY

CREST SYNDROME

MORPHEA

LINEAR SCLERODERMA

MUSCULOSKELETAL MANIFESTATIONS Arthralgia Arthritis Myalgia Myositis Muscle atrophy

ACRO-OSTEOLYSIS

GI INVOLVEMENT ESOPHAGUS

GI INVOLVEMENT STOMACH & DEODENUM

GI INVOLVEMENT COLON

PULMONARY MANIFESTATIONS Alveolitis Interstitial fibrosis Pulmonary hypertension Aspiration pneumonia Pulmonary hemorrhage Pneumothorax Lung cancer

SCLERODERMA RENAL CRISIS Hypertension Uremia Proteinuria Hematuria

CARDIAC MANIFESTATIONS MYOCARDITIS PERICARDITIS CONDUCTION DEFECT CORONARY ARTERY DISEASE