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Case Reports
. 2022 Oct 15;61(20):3095-3100.
doi: 10.2169/internalmedicine.9015-21. Epub 2022 Apr 2.

Unilateral Autoimmune Pulmonary Alveolar Proteinosis with Polymyositis-related Interstitial Lung Disease

Yutaka Muto  1 , Eri Hagiwara  1 , Tomohisa Baba  1 , Yozo Sato  1 , Masashi Sakayori  1 , Erina Tabata  1 , Akimasa Sekine  1 , Shigeru Komatsu  1 , Koji Okudela  2 , Koichi Sayama  3 , Takashi Ogura  1
Affiliations
Case Reports

Unilateral Autoimmune Pulmonary Alveolar Proteinosis with Polymyositis-related Interstitial Lung Disease

Yutaka Muto et al. Intern Med. .

Abstract

A 61-year-old patient with cystic bronchiectasis and bronchial artery hyperplasia in the left lung was diagnosed with polymyositis-related interstitial lung disease. After nine months of immunosuppressive therapy, he developed unilateral autoimmune pulmonary alveolar proteinosis (APAP) in the right lung with respiratory failure. After bronchial artery embolization to prevent massive hemoptysis, whole-lung lavage was performed using veno-venous extracorporeal membrane oxygenation. His respiratory condition improved, and he was discharged from the hospital with supplemental oxygen. Three reported cases of APAP with polymyositis-related interstitial lung disease, including the present case, were all positive for anti-glycyl tRNA synthetase antibody and were under immunosuppressive treatment.

Keywords: anti-glycyl tRNA synthetase antibody; bronchiectasis; interstitial lung disease; polymyositis; pulmonary alveolar proteinosis; whole-lung lavage.

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Conflict of interest statement

The authors state that they have no Conflict of Interest (COI).

Figures

Figure 1.
Figure 1.
a: Chest computed tomography revealed lower lung-predominant peribronchovascular ground-glass or reticular opacities in the right lung when the patient was diagnosed with polymyositis-related interstitial lung disease. Cystic bronchiectasis and thickened bronchial wall were observed in the left lung. b: On admission to our hospital, thickened reticular lines superimposed on areas of ground-glass opacities appeared only in the right lung.
Figure 2.
Figure 2.
Enhanced computed tomography of the chest revealed left bronchial artery hyperplasia.
Figure 3.
Figure 3.
Cytological findings of the whole-lung lavage fluid revealed eosinophilic globules in the Papanicolaou-stained smear (×400). Globules stained positive with Periodic Acid-Schiff were also observed (×400).
Figure 4.
Figure 4.
The reticular lines with ground-glass opacities, which had been observed on chest computed tomography on admission, improved a week after whole-lung lavage in the right lung.
Figure 5.
Figure 5.
Clinical course of the patient after being diagnosed with polymyositis-related interstitial lung disease. APAP: autoimmune pulmonary alveolar proteinosis, BAE: bronchial artery embolization, CPA: chronic pulmonary aspergillosis, IVCY: intravenous cyclophosphamide, KL-6: Krebs von den Lungen-6, LDH: lactate dehydrogenase, PM-ILD: polymyositis-related interstitial lung disease, PSL: prednisolone, SP-D: surfactant protein D, TAC: tacrolimus, VRCZ: voriconazole, WLL: whole-lung lavage
See this image and copyright information in PMC

References

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