Overview
Definition
Autoimmune disease characterized by widespread fibrosis of the skin and internal organs.
Epidemiology
Rare, more common in women.
Etiology & Risk Factors
- Autoimmune endothelial damage leading to fibroblast activation and collagen deposition
Clinical Symptoms
- Skin thickening
- Raynaud's phenomenon
- GERD
- Sclerodactyly
- Telangiectasia
Clinical Approach
Diagnosis
- Anti-centromere antibodies (CREST/Limited)
- Anti-Scl-70 antibodies (Diffuse)
Management
- Organ-specific symptomatic therapy
- Calcium channel blockers for Raynaud's
- Immunosuppressants
Complications
- Pulmonary arterial hypertension
- Interstitial lung disease
- Scleroderma renal crisis