Open AccessPulmonary veno‐occlusive disease in pulmonary Langerhans′ cell granulomatosis
Author(s) -
Hamada K.,
Teramoto S.,
Narita N.,
Yamada E,
Teramoto K,
Kobzik L
Publication year - 2000
Publication title -
european respiratory journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.021
H-Index - 241
eISSN - 1399-3003
pISSN - 0903-1936
DOI - 10.1034/j.1399-3003.2000.15b33.x
Subject(s) - medicine , autopsy , pulmonary veno occlusive disease , pathological , pulmonary fibrosis , pathology , lung , respiratory failure , pulmonary hypertension , disease , fibrosis
This report describes unusual clinical and pathological findings in a 29‐yr‐old female with pulmonary Langerhans′ cell granulomatosis (LCG). During a 7‐yr clinical course her condition deteriorated despite corticosteroid therapy, and she died of respiratory failure and pulmonary hypertension. At autopsy, there were widespread pulmonary veno‐occlusive disease (PVOD) lesions as well as abundant advanced and healed lesions of pulmonary LCG composed of multiple cysts and stellate fibrosis. The present case demonstrates that pulmonary Langerhans′ cell granulomatosis should be considered as a possible cause of pulmonary veno‐occlusive disease.