A patient with diffuse cutaneous systemic sclerosis is at highest risk for which life-threatening complication, and what antibody is most strongly associated with it?

• A Pulmonary arterial hypertension; anti-centromere antibody (ACA)
• B Scleroderma renal crisis; anti-RNA polymerase III antibody ✓
• C Interstitial lung disease; anti-Scl-70 (anti-topoisomerase I)
• D Cardiac arrhythmias; anti-U1 RNP antibody

Explanation

Scleroderma renal crisis (SRC) — abrupt onset severe hypertension, rapidly progressive renal failure, thrombotic microangiopathy — is most strongly associated with anti-RNA polymerase III antibodies (present in ~25% of diffuse SSc) and is the most feared acute life-threatening complication of diffuse SSc. Anti-Scl-70 correlates with ILD and diffuse skin disease; anti-centromere antibody correlates with limited SSc and PAH. Early ACE inhibitor use is the cornerstone of SRC treatment.

Reference: Neena Khanna Illustrated Synopsis of Dermatology & STD, 6th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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