Amyloidosis of the respiratory system: 16 patients with amyloidosis initially diagnosed ante mortem by pulmonologists
Background Ante mortem diagnosis of amyloidosis of the respiratory system is rare. Few data are available regarding clinical presentation, precursor proteins, diagnostic procedures, comorbidities, complications, and outcome. We assessed clinical features of a series of patients with amyloidosis of t...
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doaj-79cc97cf331448f49be886a401d9bc2e2020-11-25T03:40:00ZengEuropean Respiratory SocietyERJ Open Research2312-05412020-07-016310.1183/23120541.00313-201900313-2019Amyloidosis of the respiratory system: 16 patients with amyloidosis initially diagnosed ante mortem by pulmonologistsMasami Yamada0Noboru Takayanagi1Hideaki Yamakawa2Takashi Ishiguro3Tomohisa Baba4Yoshihiko Shimizu5Koji Okudela6Tamiko Takemura7Takashi Ogura8 Dept of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center, Saitama, Japan Dept of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center, Saitama, Japan Dept of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan Dept of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center, Saitama, Japan Dept of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan Dept of Diagnostic Pathology, Saitama Cardiovascular and Respiratory Center, Saitama, Japan Dept of Pathobiology, Yokohama City University Graduate School of Medicine, Yokohama, Japan Dept of Pathology, Japanese Red Cross Medical Center, Tokyo, Japan Dept of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center, Saitama, Japan Background Ante mortem diagnosis of amyloidosis of the respiratory system is rare. Few data are available regarding clinical presentation, precursor proteins, diagnostic procedures, comorbidities, complications, and outcome. We assessed clinical features of a series of patients with amyloidosis of the respiratory system in two Japanese centres. Methods Medical records of 16 patients with amyloidosis of the respiratory system were retrospectively analysed. Amyloid was diagnosed by polarisation microscopy using Congo red-stained tissue specimens and classified immunohistochemically. Results Median patient age was 71 years, and median follow-up period was 5 years. Immunoglobulin light-chain (AL)-λ amyloidosis was found in eight and AL-κ in five patients. Two patients harboured wild-type transthyretin and one harboured serum amyloid A-derived amyloid. Five different forms of amyloidosis of the respiratory system were observed: nodular pulmonary amyloidosis (seven patients), diffuse alveolar-septal amyloidosis (five), mediastinal lymph node amyloidosis (three), tracheobronchial amyloidosis (one), and pleural amyloidosis (one). One patient had diffuse alveolar-septal amyloidosis and mediastinal lymph node amyloidosis. Three of five patients with diffuse alveolar-septal amyloidosis were diagnosed by transbronchial lung biopsy as having concurrent diffuse alveolar haemorrhage or pneumocystis pneumonia. Two of three patients with mediastinal lymph node amyloidosis were diagnosed by endobronchial ultrasound-guided transbronchial needle aspiration. Conclusions Not only nodular pulmonary amyloidosis, diffuse alveolar-septal amyloidosis, and tracheobronchial amyloidosis but also mediastinal lymph node amyloidosis and pleural amyloidosis should be considered in the differential diagnosis of amyloidosis of the respiratory system. Useful diagnostic methods include transbronchial lung biopsy for diffuse alveolar-septal amyloidosis and endobronchial ultrasound-guided transbronchial needle aspiration for mediastinal lymph node amyloidosis.http://openres.ersjournals.com/content/6/3/00313-2019.full |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Masami Yamada Noboru Takayanagi Hideaki Yamakawa Takashi Ishiguro Tomohisa Baba Yoshihiko Shimizu Koji Okudela Tamiko Takemura Takashi Ogura |
spellingShingle |
Masami Yamada Noboru Takayanagi Hideaki Yamakawa Takashi Ishiguro Tomohisa Baba Yoshihiko Shimizu Koji Okudela Tamiko Takemura Takashi Ogura Amyloidosis of the respiratory system: 16 patients with amyloidosis initially diagnosed ante mortem by pulmonologists ERJ Open Research |
author_facet |
Masami Yamada Noboru Takayanagi Hideaki Yamakawa Takashi Ishiguro Tomohisa Baba Yoshihiko Shimizu Koji Okudela Tamiko Takemura Takashi Ogura |
author_sort |
Masami Yamada |
title |
Amyloidosis of the respiratory system: 16 patients with amyloidosis initially diagnosed ante mortem by pulmonologists |
title_short |
Amyloidosis of the respiratory system: 16 patients with amyloidosis initially diagnosed ante mortem by pulmonologists |
title_full |
Amyloidosis of the respiratory system: 16 patients with amyloidosis initially diagnosed ante mortem by pulmonologists |
title_fullStr |
Amyloidosis of the respiratory system: 16 patients with amyloidosis initially diagnosed ante mortem by pulmonologists |
title_full_unstemmed |
Amyloidosis of the respiratory system: 16 patients with amyloidosis initially diagnosed ante mortem by pulmonologists |
title_sort |
amyloidosis of the respiratory system: 16 patients with amyloidosis initially diagnosed ante mortem by pulmonologists |
publisher |
European Respiratory Society |
series |
ERJ Open Research |
issn |
2312-0541 |
publishDate |
2020-07-01 |
description |
Background
Ante mortem diagnosis of amyloidosis of the respiratory system is rare. Few data are available regarding clinical presentation, precursor proteins, diagnostic procedures, comorbidities, complications, and outcome. We assessed clinical features of a series of patients with amyloidosis of the respiratory system in two Japanese centres.
Methods
Medical records of 16 patients with amyloidosis of the respiratory system were retrospectively analysed. Amyloid was diagnosed by polarisation microscopy using Congo red-stained tissue specimens and classified immunohistochemically.
Results
Median patient age was 71 years, and median follow-up period was 5 years. Immunoglobulin light-chain (AL)-λ amyloidosis was found in eight and AL-κ in five patients. Two patients harboured wild-type transthyretin and one harboured serum amyloid A-derived amyloid. Five different forms of amyloidosis of the respiratory system were observed: nodular pulmonary amyloidosis (seven patients), diffuse alveolar-septal amyloidosis (five), mediastinal lymph node amyloidosis (three), tracheobronchial amyloidosis (one), and pleural amyloidosis (one). One patient had diffuse alveolar-septal amyloidosis and mediastinal lymph node amyloidosis. Three of five patients with diffuse alveolar-septal amyloidosis were diagnosed by transbronchial lung biopsy as having concurrent diffuse alveolar haemorrhage or pneumocystis pneumonia. Two of three patients with mediastinal lymph node amyloidosis were diagnosed by endobronchial ultrasound-guided transbronchial needle aspiration.
Conclusions
Not only nodular pulmonary amyloidosis, diffuse alveolar-septal amyloidosis, and tracheobronchial amyloidosis but also mediastinal lymph node amyloidosis and pleural amyloidosis should be considered in the differential diagnosis of amyloidosis of the respiratory system. Useful diagnostic methods include transbronchial lung biopsy for diffuse alveolar-septal amyloidosis and endobronchial ultrasound-guided transbronchial needle aspiration for mediastinal lymph node amyloidosis. |
url |
http://openres.ersjournals.com/content/6/3/00313-2019.full |
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