Open AccessPulmonary alveolar proteinosis after lung transplantation
Author(s) -
Divithotawela Chandima,
Apte Simon H.,
Tan Maxine E.,
De Silva Tharushi A.,
Chambers Daniel C.
Publication year - 2020
Publication title -
respirology case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.304
H-Index - 9
ISSN - 2051-3380
DOI - 10.1002/rcr2.566
Subject(s) - pulmonary alveolar proteinosis , bronchoalveolar lavage , medicine , pathology , lung , lipofuscin , lung transplantation , pulmonary function testing , lung biopsy , biopsy
We report the case of a 69‐year‐old man five‐month post double lung transplant for idiopathic pulmonary fibrosis (IPF) who presented with progressive breathlessness, loss of lung function, and diffuse ground glass shadowing on the chest computed tomography. Transbronchial lung biopsy revealed foamy macrophages, hyperplasia of type II pneumocytes, and eosinophilic material in the alveolar space. Video thoracic lung biopsy was performed, and histology confirmed pulmonary alveolar proteinosis. Anti‐granulocyte‐macrophage colony‐stimulating factor (GM‐CSF) antibodies were negative. Bilateral sequential whole lung lavage (WLL) was performed. Lavage fluid recovered during WLL was notably dark brown in colour and upon analysis was shown to contain heavily oxidized protein (lipofuscin), giant lipofuscin‐engorged macrophages, and a highly pro‐inflammatory gene expression profile. Following WLL, the patient's symptoms, lung function, and radiology appearance improved. His repeat bronchoalveolar lavage (BAL) fluid analysis showed reduced lipofuscin and normalized macrophage size and gene expression.