Autoimmune pulmonary alveolar proteinosis with features similar to nonspecific interstitial pneumonia

Abstract

A 58-year-old woman with cough and dyspnea who was suspected of having idiopathic interstitial pneumonia had been treated with corticosteroids and cyclosporine, but the symptoms had worsened. There were no findings to suspect pulmonary alveolar proteinosis (PAP) in the bronchoalveolar lavage fluid, 17 months after the start of treatment. The transbronchial lung biopsy specimens showed eosinophilic bodies that strongly stained with periodic acid-Schiff staining. Anti-granulocyte macrophage colony-stimulating factor (anti-GM-CSF) antibodies were detected in her serum. We diagnosed the patient with autoimmune PAP. Thus, we present a rare case of PAP presenting atypical radiological images and bronchoalveolar lavage fluid findings.

Keywords: Anti-GM-CSF antibody; Autoimmune pulmonary alveolar proteinosis; Bronchoalveolar lavage fluid; Ground-glass opacities; Nonspecific interstitial pneumonia.

Fig. 1

Computed tomography images (A) During the first visit, we observed bilateral ground-glass opacities with significant distribution in the peripheral regions. (B) Nine months after the first visit, the abnormal shadows had expanded to neighboring regions. (C) Seventeen months after the first visit, the abnormal shadows had spread in the center.

Fig. 2

Transbronchial lung biopsy specimens stained with (A) hematoxylin and eosin and (B) periodic acid-Schiff staining. The biopsy specimens contained eosinophilic bodies that stained strongly with periodic acid-Schiff staining.