Scleroderma: The Daily PANCE Blueprint

Scleroderma: The Daily PANCE Blueprint

Which of the following antibodies is most likely positive in someone with limited cutaneous systemic sclerosis?

A. anticentromere antibody
B. antitopoisomerase-1 antibody
C. anti-Jo-1 antibody
D. anti-cardiolipin antibody
E. anti-dsDNA antibody

Answer and topic summary

The answer is A. anticentromere antibody

Systemic sclerosis is a chronic connective tissue disease defined by excessive collagen deposition in skin and organs. It leads to diffuse microvascular damage. Defining features of this disease include Raynaud phenomenon, hardening of the skin (aka scleroderma), pulmonary fibrosis, and pulmonary arterial hypertension. The are two main subsets of SS: limited cutaneous (60% of patients) and diffuse cutaneous. The limited cutaneous subset has a variant known by many as CREST syndrome (Calcinosis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, telangiectasis). Most patients will have a positive antinuclear antibody. Those with limited cutaneous disease will likely be positive for anticentromere antibody, whereas those with diffuse cutaneous may have antitopoisomerase-1 antibody (anti-Scl-70). Treatment is geared towards the organ-specific complications of systemic sclerosis.

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