1. Scleroderma

2. Scleroderma
Generalized disorder of connective tissue affecting the skin , connective tissue and vasculature
Peak age yrs age
Female 4 : male 1
Limited disease , diffuse disease , CREST syndrome ( calcinosis , Raynaud’s , esophageal involvement , sclerodactyly and talangiectasia _)

3. Calcinosis

4. Calcinosis

5. Calcification

6. Marked Calcification

7. Marked Calcification involving the muscle

8. Calcinosis with nodules

9. Raynaud’s phenomenon

10. Esophageal dilatation

11. Early Sclerodactyly

12. Early sclerodactyly

13. Late Sclerodactyly

14. Talangiectasia

15. Etiology and pathogenesis
Unknown etiology ( no genetic , geographical or race association )
Environmental factors as exposure to silica , dust , vinyl chloride , hypoxia resins and trichloroethylene )
Skin infiltration by T lymphocyte and abnormal fibroblast activation lead to increased production of collagen type I which results in thickening , tightening and induration of the skin (sclerodactyly )

16. Etiology and pathogenesis
Arterial and arteriolar narrowing due to initial proliferation and vessel wall inflammation
Endothelial injury causes release of vasoconstrictor and platelet activation

17. Clinical features Sclerodactyly proximal to MCP Js
Most patients are ANA positive
30 % of diffuse disease and 60 % of limited disease have Ab to topoisomerase I and centromere respectively

18. Clinical features Cutaneous changes :
Raynaud’s phenomenon is universal
Non pitting oedema of the fingers and the flexor tendon sheath , then the skin become taut and shiny and distal skin creases disappear
Erythema and tortuous dilatation of capillary loops in nail fold bed

19. Non pitting oedema of the fingers

20. Erythema and tortuous dilatation of capillary loops in nail fold bed

21. Taut skin and shiny

22. Clinical features Cutaneous changes :
Skin involvement restricted to sites distal to the elbow or knee is classified as limited disease
Involvement proximal to the knee and elbow and on the trunk is classified as diffuse disease
Tissue ischemia leading to skin ulceration over pressure area , pulp atrophy of the finger tips

23. Tissue ischemia , skin ulceration and pulp atrophy

24. Digital loss

25. Perioral scleroderma with furrowing

26. Perioral scleroderma with furrowing

27. Clinical features Musculoskeletal features :
Arthralgia , morning stiffness , flexor tenosynovitis
Restricted hand function
Erosive arthropathy is unknown
Muscle weakness and wasting are usually due to myositis

28. Clinical features Gastrointestinal features
Reflux with erosive eosophagitis ( Dysphagia and odynophagia
Early satiety and occasionally outlet obstruction
Recurrent occult upper GI bleeding may indicate watermelon stomach which occur in 20% of patients

29. erosive eosophagitis

30. Clinical features Gastrointestinal features
Malabsorption due to bacterial overgrowth and intermittent bloating , pain or constipation
Autonomic neuropathy may cause pseudo – obstruction

31. Clinical features Cardiorespiratory features :
Fibrosing alveolitis in patient with diffuse disease
Pulmonary hypertension more in limited than diffuse disease

32. Clinical features Renal features :
Hypertensive renal crisis is the main cause of death ( presented with malignant hypertension and renal failure )

33. Management and Prognosis
Hypertensive renal crisis is treated by angiotensin converting enzyme inhibitors
Avoidance of peripheral cold exposure
Calcium antagonist (nifedipine , amlodipine ) or angiotensin II receptor antagonist ( valsartan ) may be used for Raynaud’s symptoms

34. Management and Prognosis
For digital ischemia intermittent infusion of prostacyclin may be helpful
Steroids and cytotoxic drugs are used in myositis or alveolitis D- penicillamine and Interferon gamma may be used in skin

35. Mixed connective tissue disease
His is an overlap connective tissue disease with features of SLE , scleroderma and myositis
Synovitis and edema of the hands in combination of Raynaud’s phenomenon and muscle pain / weakness
Most patients have anti – ribonucleoprotein (RNP ) antibodies