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Scleroderma

Scleroderma. By…….. Manohar Joshi Medicine Resident. Scleroderma. Also called as systemic sclerosis. Occurs due to fibroblast activation. There is excessive fibrosis. Its cause is unknown , but it occurs due to abnormal activation of immune system and microvascular injury.

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Scleroderma

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  1. Scleroderma By……..Manohar Joshi Medicine Resident

  2. Scleroderma Also called as systemic sclerosis. Occurs due to fibroblast activation. There is excessive fibrosis. Its cause is unknown , but it occurs due to abnormal activation of immune system and microvascular injury. Along with skin , GIT , lung , heart , kidney get involved.

  3. CLASSIFICATION: • Systemic Sclerosis • Diffuse scleroderma • Limited scleroderma(CREST syndrome) • Sine scleroderma(visceral organ involvement) • Localized scleroderma • Morphea(single/multiple plaques of skin indurations) • Linear scleroderma(only face and extremity) • En coup de sabre • Others • Overlap syndrome • Mixed connective tissue disorder • Eosinophilicfascitis • Eosinophiliamyalgia syndrome

  4. Etiology • More common in females 3 : 1 • 3rd to 5th decade. • More common in monozygotic twins. • CMV is mediated through direct vascular injury • Parvo virus B 19. • Drugs : vinyl chloride, pentazocine(non-narcotic agent) bleomycin(anti cancer)

  5. Pathology Two types of response • Vascular • Immune

  6. Vascular response • It involves raynaud’s phenomenon. • Leads to capillary vasospasm of extremites. • Cold (sudden vasospasm) • Blue (decreased blood supply) • Red (reactive vasodilatation)

  7. Immune response

  8. Pathology of organs involved • Skin thin epidermis over compact bundle of collagen • GIT Lower 2/3rd of the oesophagus is involved. Thin mucosa and increased collagen in lamina propria , submucosa , and serosa. • Lung diffuse interstitial fibrosis , thickening of alveolar membrane, peribronchial and pleural fibrosis. • Musculoskeletal arthritis, lymphocytic infiltration, degeneration of muscle fibers , arterioles thickened

  9. Heart Degeneration of myocardial fibers, A V conduction defects with arrythmias. • Kidney intimal hyperplasia of interlobular arteries , fibrinoid necrosis , thickening of glomerular basement membrane. • Other primary biliary cirrosis and auto immune thyroiditis.

  10. Clinical manifestations: • Face : mask like facies. Pinched up nose fish – mouth (not able to open the mouth) , shiny skin. • Hands : Skin is very taut and cant be pinched up. Symmetrical swelling of fingers. pulp atrophy telengactesia nail fold thrombi, digital infarct and ulcerations

  11. GIT : Heart burns , reflux oesophagitis Dysphagia Mal absorption Chronic constipation. • Musculoskeletal : Poly arthritis, Leathery crepitations , Carpal tunnel syndrome. • Lung : Dyspnea and dry cough , Bilateral basilar rales , Interstitial lung disease , PH. • Heart :Pericarditis , heart blocks Arrhythmias and LVF. • Renal : Malignant HT and Progressive Renal Failure

  12. Other : Dry mouth or eyes Hypothyroidism Sjogren’s syndrome Biliary cirrhosis.

  13. Laboratory Findings : • Anemia . • Vitamin B12 deficiency. • Cryoglobulin present in serum. • ANA shows anti topoisomerase 1 • Nail – fold capillary scopy. • Sereal PFT • Oesophageal manomatory

  14. Treatment • Raynaud’s phenomenon 1) methyldopa , reserine, phenoxybenzamine , prazosin 2) calcium channel blocker like nifedepine , nifedepine sustain release , diltiazem 3) ketaserin 40mg TDS 4) fluroxetine 20mg/day 5) pentoxyfyline:40mg TDS improve reperfusion

  15. D-penicillamine : low dose is required 125mg • Methotrexate show improvement in skin lesion • Cyclophosphamide has also shown some improvement skin lesion and systemic manifestation • Stem cell transplantation • Anti platelet • Dipyridamole 200 to 400mg,it decrease platelet adhesion to vessels • Colchicine , INF-α, INF- γ, recombinant human relaxin • -

  16. Proton pump inhibitor - like ranitidine, cimitidine can be used in reflux oesophagitis / heart burn. • Pilocarpine - as it increases secretion in condition like dry mouth and dry eye. • Cervical and digital sympathectomy can be helpful in vascular injury.

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