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Chronic thromboembolic pulmonary hypertension — ABIM Board MCQ

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HardPulmonary/Critical CareChronic thromboembolic pulmonary hypertensionABIM Board

A 36-year-old woman has diffuse thin-walled lung cysts, a renal angiomyolipoma, elevated VEGF-D, and progressive exertional dyspnea. FEV1 has fallen to 62% predicted over one year despite bronchodilator therapy. Which disease-modifying treatment is most appropriate?

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Correct answer: A — Start sirolimus with trough-level and toxicity monitoring

The best answer is “Start sirolimus with trough-level and toxicity monitoring”. The phenotype is lymphangioleiomyomatosis with abnormal and declining lung function. ATS/JRS guidance recommends sirolimus, which can stabilize lung function and improve selected manifestations. Monitoring includes drug concentrations, cytopenias, hepatic effects, lipids, infection risk, and mucosal toxicity. Doxycycline and hormonal manipulation are not recommended disease-modifying strategies, and waiting permits avoidable physiologic loss.

Reference: ATS/JRS Guideline for Lymphangioleiomyomatosis: https://www.thoracic.org/statements/guideline-implementation-tools/lam-diagnosis-and-mgmt.php