Which pulmonary complication is most commonly the cause of death in limited cutaneous systemic sclerosis (lcSSc)?

• A Pleural effusion
• B Interstitial lung disease (ILD) / usual interstitial pneumonia
• C Pulmonary arterial hypertension (PAH) ✓
• D Pulmonary embolism from antiphospholipid antibodies

Explanation

In limited cutaneous SSc (anticentromere antibody-associated), pulmonary arterial hypertension is the leading cause of death, occurring late in the disease course (often after 10-15 years). ILD (particularly NSIP pattern) is the most common pulmonary cause of death in diffuse cutaneous SSc (anti-Scl-70/anti-topoisomerase-I-associated). This distinction is high-yield: lcSSc → PAH is the primary pulmonary killer; dcSSc → ILD is. PAH in lcSSc is managed with endothelin receptor antagonists, PDE5 inhibitors, or prostacyclin analogues.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.