Treatment of Pulmonary Hemangiomatosis with Recombinant Interferon Alfa-2a

TLDR

Pulmonary hemangiomatosis is a rare, fatal disease of diffuse microvascular proliferation that rapidly progresses to pulmonary hypertension or bleeding, mainly affecting children and young adults, and is usually diagnosed post‑mortem; prior treatments with corticosteroids and cyclophosphamide have failed to achieve success. The authors report a case of pulmonary hemangiomatosis presenting with digital clubbing and bilateral interstitial pulmonary disease. Interferon alfa‑2a therapy was initiated after a decline in exercise tolerance and continued for 14 months. No additional information.

Abstract

PULMONARY hemangiomatosis is a rare, fatal disorder characterized by diffuse microvascular proliferation within the lung. The disease most often affects children and young adults and usually progresses rapidly, causing death from pulmonary hypertension or bleeding. Most cases have been diagnosed at autopsy. Corticosteroids and cyclophosphamide have been used for this condition, but no successful therapy has been described.We describe a patient with pulmonary hemangiomatosis who presented with digital clubbing and bilateral interstitial pulmonary disease. After a decline in the patient's exercise tolerance, therapy with interferon alfa-2a was initiated and has been continued for 14 months at the time of . . .