Scleroderma by aseem

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Scleroderma by aseem

  1. 1. SYSTEMIC SCLEROSIS
  2. 2. INTRODUCTION • Syn SCLERODERMA • Gk: Sclero – Hardening / fibrosis ; Derma – Skin • Definition: a Clinically diverse, Multisystem disorder characterized by vascular abnormalities, connective tissue sclerosis and atrophy and Hallmark Autoantibodies
  3. 3. INTRODUCTION • • • • F : M = 5 : 1 to 14 : 1 30 – 50 years Prevalence 1-2 / 10000 Highest case-specific Mortality Rate amongst AI diseases
  4. 4. CLASSIFICATION
  5. 5. CLASSIFICATION Limited SSc - Cutaneous, affects Face, Hands, Arms - erst CREST SYN (PAH) Diffuse SSc - Organ Involvement viz Heart / Lung / Esophagus / Kidneys Systemic sine Sclerosis – No Skin Changes Localized - Morphea / Linear Scleroderma – No organ involvement
  6. 6. AETIOPATHOGENESIS • Exact etiology Unknown • Endothelial cell injury • Genetic factors – HLA B8 / CXCR2 / TNF-A / IL-10 / ACE / TGF-B1 • Autoimmunity characterized by Hallmark ABs
  7. 7. SSc Hallmark ABs
  8. 8. PATHOGENESIS • xx
  9. 9. Diagnostic criteria • ACR (1980) vs ACR-EULAR (2013) • Major: 1. Symmetric skin thickening proximal to MCP or MTP joints (09) • Minor: 1. Sclerodactyly (04) 2. Digital pitted scars/ loss of finger tip pulp (MIZUTANI SIGN) (03) 3. Bibasilar pulmonary fibrosis – linear / nodular in absence of Primary Lung Disease (02)
  10. 10. • 1. 2. 3. Additional minor criteria: Raynaud’s phenomenon (03) Nail fold capillary changes (‘Drop-Out’ Sign) (02) SSc selective antibodies (03) • 01 major or 02 minor criteria (09 or >09 with Telangiectasia / Edema Acral 02 each) • • 97 % sensitive & 98 % specific (ACR-EULAR) 73 % Sn 76% Sp (ACR / ARA standalone)
  11. 11. CLINICAL FEATURES LcSSc -Long history of Raynaud’s phenomenon -Limited skin involvement (peripheral only) -Calcification, telangiectasia, high frequency of visceral disease, late onset of PAH - Low frequency of Scleroderma Renal Crisis -Capillary dilatation visible in nail folds - CREST Syndrome -Anticentromere antibody-positive
  12. 12. CLINICAL FEATURES • DcSSc -Short interval (< 1 year) between the onset of Raynaud’s phenomenon and the development of skin changes -Truncal and peripheral skin involvement (Proximal) -Pulmonary fibrosis/ ILD, renal crisis, gastrointestinal disease, myocardial involvement -Capillary drop-out visible in nail folds -Scl-70 / anti RNAPIII antibody-positive -Anticentromere antibody-negative
  13. 13. CLINICAL FEATURES • Overlap SSc Scleroderma features + DM / PM / SLE / SJOGREN’s / VASCULITIS / POLYARTRITIS ANA-positive (Nucleolar / Speckled) SSS Ro / La + ds DNA / Anti-Sm + RA factor +
  14. 14. Clinical features • Raynaud’s phenomenon
  15. 15. • Nail bed changes – decrease in capillary loops – ‘Drop Out Sign’
  16. 16. • Skin changes: 3 stages:1. Oedematous phase – puffy fingers 2. Indurative phase – tight, hidebound skin 3. Atrophic phase – softened skin
  17. 17. OEDEMATOUS PHASE
  18. 18. INDURATIVE PHASE
  19. 19. DYSPIGMENTATION • Salt and Pepper appearance with areas of hyperpigmentation alternating with hypopigmentation (Pseudo-Vitiligo)
  20. 20. CALCINOSIS
  21. 21. SYSTEMIC FEATURES
  22. 22. SYSTEMIC SYMPTOMS • Z
  23. 23. Thank you

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