Abstract | BACKGROUND:
Pulmonary alveolar proteinosis (PAP) is a diffuse lung disease characterized by the abnormal accumulation of surfactant-like material within the alveolar spaces and distal bronchioles. If high-resolution computed tomography (HRCT) indicates the presence of PAP, a definitive diagnosis of PAP is established when consistent pathological findings are obtained. Herein, we retrospectively studied the yield and safety of bronchofiberscopy in the diagnosis of PAP. METHODS: One hundred and fifty consecutive patients with PAP were prospectively registered in the PAP cohort database of the National Hospital Organization Kinki-Chuo Chest Medical Center between January 1991 and December 2018. We examined 86 patients who underwent bronchofiberscopy with bronchoalveolar lavage (BAL) and transbronchial lung forceps biopsy (TBLB). RESULTS: The patients included 56 men and 30 women, with a median age of 57 years. All patients had autoimmune PAP, and the median level of anti- granulocyte-macrophage colony-stimulating factor ( GM-CSF) autoantibodies was 42.8 μg/mL. The diagnostic yield was 90.7% (78/86) with BAL and 81.4% (70/86) with TBLB. The combination of BAL and TBLB increased the yield to 98.8%. Age, disease severity score, and frequency of traction bronchiectasis on HRCT were significantly different between the TBLB-positive and TBLB-negative groups. No patient developed serious complications due to bronchofiberscopy; TBLB-related complications included pneumothorax (3.5%) and minimal bleeding (7.0%). CONCLUSIONS: Bronchofiberscopy, in combination with BAL and TBLB, is an effective and safe method for the diagnosis of PAP, with a yield of 98.8%.
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Authors | Koji Azuma, Takayuki Takimoto, Takahiko Kasai, Masaki Hirose, Kazuyoshi Hatsuda, Chikatoshi Sugimoto, Toru Arai, Masanori Akira, Yoshikazu Inoue |
Journal | Respiratory investigation
(Respir Investig)
Vol. 59
Issue 6
Pg. 757-765
(Nov 2021)
ISSN: 2212-5353 [Electronic] Netherlands |
PMID | 33967014
(Publication Type: Journal Article)
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Copyright | Copyright © 2021 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved. |
Chemical References |
- Autoantibodies
- Granulocyte-Macrophage Colony-Stimulating Factor
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Topics |
- Autoantibodies
- Bronchoalveolar Lavage
- Female
- Granulocyte-Macrophage Colony-Stimulating Factor
- Humans
- Male
- Middle Aged
- Pulmonary Alveolar Proteinosis
(diagnosis)
- Retrospective Studies
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