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

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

4. 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

5. 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

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

7. CLINICAL MANIFESTATION

8. 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

9. 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

10. RAYNOUD’S PHENOMENON

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

12. EDEMATOUS PHASE

13. INDURATIVE PHASE

14. SCLEROTIC PHASE

15. CALCIFICATION

16. PITTING SCAR

17. CAPILAROSCOPY

18. CREST SYNDROME

19. MORPHEA

20. LINEAR SCLERODERMA

21. MUSCULOSKELETAL MANIFESTATIONS
Arthralgia
Arthritis
Myalgia
Myositis
Muscle atrophy

22. ACRO-OSTEOLYSIS

23. GI INVOLVEMENT
ESOPHAGUS

24. GI INVOLVEMENT
STOMACH & DEODENUM

25. GI INVOLVEMENT
COLON

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

29. SCLERODERMA RENAL CRISIS
Hypertension
Uremia
Proteinuria
Hematuria

30. CARDIAC MANIFESTATIONS
MYOCARDITIS
PERICARDITIS
CONDUCTION DEFECT
CORONARY ARTERY DISEASE