1. SYSTEMIC SCLEROSIS

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. INTRODUCTION
•
•
•
•
F : M = 5 : 1 to 14 : 1
30 – 50 years
Prevalence 1-2 / 10000
Highest case-specific Mortality Rate amongst
AI diseases

4. CLASSIFICATION

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. 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. SSc Hallmark ABs

9. PATHOGENESIS
• xx

10. 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)

11. •
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)

12. 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

13. 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

14. 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 +

15. Clinical features
• Raynaud’s phenomenon

16. • Nail bed changes – decrease in capillary loops
– ‘Drop Out Sign’

17. • Skin changes:
3 stages:1. Oedematous phase – puffy fingers
2. Indurative phase – tight, hidebound skin
3. Atrophic phase – softened skin

18. OEDEMATOUS PHASE

19. INDURATIVE PHASE

20. DYSPIGMENTATION
• Salt and Pepper appearance with areas of
hyperpigmentation alternating with
hypopigmentation (Pseudo-Vitiligo)

21. CALCINOSIS

24. SYSTEMIC FEATURES

25. SYSTEMIC SYMPTOMS
• Z

26. Thank you