Systemic Sclerosis (Scleroderma)
Multi-system autoimmune disease characterized by vasculopathy and progressive fibrosis of skin and internal organs
3 Subtypes of Scleroderma (SSc):
• Limited cutaneous systemic sclerosis (LcSSc) - distal skin thickening, distal to elbows/wrists and knees
• Diffuse systemic sclerosis (DcSSc; diffuse) - skin thickening extends proximally to arms, thighs, and/or trunk
• Systemic Sclerosis sine scleroderma (ssSSc) - fibrosing internal organ involvement without skin thickening
Signs and Symptoms:
• Cardiac: myocardial fibrosis, pericarditis
• Hepatic: PBC (primary biliary cholangitis) overlap
• Gastrointestinal: GERD, dysmotility, SIBO, GAVE, pseudoobstruction
• Cutaneous ("salt and pepper" skin changes): skin thickening, sclerodactyly, telangiectasias, poikiloderma, calcinosis
• Pulmonary: ILD (interstitial lung disease), PAH (pulmonary arterial HTN)
• Renal: scleroderma renal crisis (SRC)
• Musculoskeletal: joint involvement, acro-osteolysis, myositis overlap
• Vascular: Raynaud's, digital ulcers
Mithu Maheswaranathan, MD @MithuRheum
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