Systemic sclerosis (symptoms, diagnosis, treatment, classification)
1. Big picture
Systemic sclerosis (SSc), or scleroderma, is a chronic systemic autoimmune disease characterized by a triad:
microvascular injury + immune activation + fibrosis of skin and internal organs
Classic exam pattern:
Raynaud phenomenon + puffy fingers/sclerodactyly + abnormal nailfold capillaries + anti-centromere or anti-Scl-70 antibodies + esophageal dysmotility/lung disease.
The main causes of death are usually interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). The emergency you must never miss is scleroderma renal crisis.
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