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Open Access 08-04-2024 | Respiratory Medicine | Case Report

A case of autoimmune pulmonary alveolar proteinosis during the course of treatment of rapidly progressive interstitial pneumonia associated with anti-MDA5 antibody-positive dermatomyositis

Authors: Masakiyo Yatomi, Keiichi Akasaka, Shintaro Sato, Mizuki Chida, Mio Kanbe, Hiru Sawada, Itaru Yokota, Ikuo Wakamatsu, Sohei Muto, Mari Sato, Kochi Yamaguchi, Yosuke Miura, Hiroaki Tsurumaki, Reiko Sakurai, Kenichiro Hara, Yasuhiko Koga, Noriaki Sunaga, Hideaki Yamakawa, Hidekazu Matsushima, Sahori Yamazaki, Yukie Endo, Sei-ichiro Motegi, Takeshi Hisada, Toshitaka Maeno

Published in: BMC Pulmonary Medicine | Issue 1/2024

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Abstract

Background

Autoimmune pulmonary alveolar proteinosis (APAP) is a diffuse lung disease that causes abnormal accumulation of lipoproteins in the alveoli; however, its pathogenesis remains unclear. Recently, APAP cases have been reported during the course of dermatomyositis. The combination of these two diseases may be coincidental; however, it may have been overlooked because differentiating APAP from a flare-up of interstitial pneumonia associated with dermatomyositis is challenging. This didactic case demonstrates the need for early APAP scrutiny.

Case presentation

A 50-year-old woman was diagnosed with anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatitis and interstitial pneumonia in April 2021. The patient was treated with corticosteroids, tacrolimus, and cyclophosphamide pulse therapy for interstitial pneumonia complicated by MDA5 antibody-positive dermatitis, which improved the symptoms and interstitial pneumonia. Eight months after the start of treatment, a new interstitial shadow appeared that worsened. Therefore, three additional courses of cyclophosphamide pulse therapy were administered; however, the respiratory symptoms and interstitial shadows did not improve. Respiratory failure progressed, and 14 months after treatment initiation, bronchoscopy revealed turbid alveolar lavage fluid, numerous foamy macrophages, and numerous periodic acid–Schiff-positive unstructured materials. Blood test results revealed high anti-granulocyte–macrophage colony-stimulating factor (GM-CSF) antibody levels, leading to a diagnosis of APAP. The patient underwent whole-lung lavage, and the respiratory disturbance promptly improved. Anti-GM-CSF antibodies were measured from the cryopreserved serum samples collected at the time of diagnosis of anti-MDA5 antibody-positive dermatitis, and 10 months later, both values were significantly higher than normal.

Conclusions

This is the first report of anti-MDA5 antibody-positive dermatomyositis complicated by interstitial pneumonia with APAP, which may develop during immunosuppressive therapy and be misdiagnosed as a re-exacerbation of interstitial pneumonia. In anti-MDA5 antibody-positive dermatomyositis, APAP comorbidity may have been overlooked, and early evaluation with bronchoalveolar lavage fluid and anti-GM-CSF antibody measurements should be considered, keeping the development of APAP in mind.
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Metadata
Title
A case of autoimmune pulmonary alveolar proteinosis during the course of treatment of rapidly progressive interstitial pneumonia associated with anti-MDA5 antibody-positive dermatomyositis
Authors
Masakiyo Yatomi
Keiichi Akasaka
Shintaro Sato
Mizuki Chida
Mio Kanbe
Hiru Sawada
Itaru Yokota
Ikuo Wakamatsu
Sohei Muto
Mari Sato
Kochi Yamaguchi
Yosuke Miura
Hiroaki Tsurumaki
Reiko Sakurai
Kenichiro Hara
Yasuhiko Koga
Noriaki Sunaga
Hideaki Yamakawa
Hidekazu Matsushima
Sahori Yamazaki
Yukie Endo
Sei-ichiro Motegi
Takeshi Hisada
Toshitaka Maeno
Publication date
08-04-2024
Publisher
BioMed Central
Published in
BMC Pulmonary Medicine / Issue 1/2024
Electronic ISSN: 1471-2466
DOI
https://doi.org/10.1186/s12890-024-02989-9

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