• Internal medicine · Nov 2024

    Case Reports

    Secondary Pulmonary Alveolar Proteinosis Development during the Treatment for Anti-aminoacyl-tRNA Synthetase Antibody-positive Interstitial Lung Disease.

    • Suzuka Matsuoka, Keiichi Fujiwara, Yuki Takigawa, Saki Ito, Sho Mitsumune, Keisuke Shiraha, Mayu Goda, Tomoyoshi Inoue, Miho Fujiwara, Eri Nakamura, Hiromi Watanabe, Kenichiro Kudo, Akiko Sato, Ken Sato, and Takuo Shibayama.
    • Department of Respiratory Medicine, NHO Okayama Medical Center, Japan.
    • Intern. Med. 2024 Nov 1; 63 (21): 297129762971-2976.

    AbstractA 70-year-old woman with anti-aminoacyl-tRNA synthetase (ARS) antibody-positive interstitial lung disease (ARS-ILD) received daily medications and regular cyclophosphamide cycles for recurring exacerbations. Approximately four years after immunosuppression initiation, the patient was admitted for progressive dyspnea on exertion. Chest computed tomography (CT) findings were suggestive of acute exacerbation. Despite intensified immunosuppressive treatment, the radiographic findings worsened, and serum Krebs von den Lungen-6 (KL-6) levels increased. A bronchoalveolar lavage fluid (BALF) examination revealed amorphous globules and alveolar macrophages with eosinophilic granules. Owing to negative anti-granulocyte-macrophage colony-stimulating factor antibody tests, a diagnosis of secondary pulmonary alveolar proteinosis (PAP) was established.

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