View
564
Download
3
Category
Preview:
Citation preview
SYSTEMIC SCLEROSIS
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
INTRODUCTION
• F : M = 5 : 1 to 14 : 1• 30 – 50 years• Prevalence 1-2 / 10000• Highest case-specific Mortality Rate amongst
AI diseases
CLASSIFICATION
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
AETIOPATHOGENESIS
• Exact etiology Unknown• Endothelial cell injury• Genetic factors – HLA B8 / CXCR2 / TNF-A /
IL-10 / ACE / TGF-B1• Autoimmunity characterized by Hallmark ABs
SSc Hallmark ABs
PATHOGENESIS
• xx
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)
• Additional minor criteria:1. Raynaud’s phenomenon (03)2. Nail fold capillary changes (‘Drop-Out’ Sign) (02)3. 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)
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
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
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 +
Clinical features • Raynaud’s phenomenon
• Nail bed changes – decrease in capillary loops – ‘Drop Out Sign’
• Skin changes: 3 stages:-
1. Oedematous phase – puffy fingers2. Indurative phase – tight, hidebound skin3. Atrophic phase – softened skin
OEDEMATOUS PHASE
INDURATIVE PHASE
DYSPIGMENTATION
• Salt and Pepper appearance with areas of hyperpigmentation alternating with hypopigmentation (Pseudo-Vitiligo)
CALCINOSIS
SYSTEMIC FEATURES
SYSTEMIC SYMPTOMS
• Z
Thank you
Recommended