In systemic sclerosis (SSc), which autoantibody is MOST strongly associated with scleroderma renal crisis (SRC) and mandates urgent ACE inhibitor use?

• A Anti-Scl-70 (anti-topoisomerase I) antibody
• B Anti-centromere antibody (ACA)
• C Anti-RNA polymerase III antibody ✓
• D Anti-PM-Scl antibody

Explanation

Anti-RNA polymerase III antibody is strongly associated with scleroderma renal crisis — a life-threatening complication of diffuse cutaneous SSc involving malignant hypertension and rapidly progressive oliguric renal failure. Scleroderma renal crisis has an early peak (<5 years of disease) and its treatment mandates ACE inhibitors (captopril) regardless of creatinine level. Anti-Scl-70 is linked to ILD and diffuse disease; anti-centromere to CREST and pulmonary hypertension; anti-PM-Scl to overlap myositis syndrome.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.