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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