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

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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

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