PMC7170098_01
Histopathology of the lung obtained by transbronchial biopsy shows accumulation of periodic acid Schiff-positive materials in the alveolar spaces
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F1
Bronchoalveolar lavage, transbronchial lung biopsy (Figure 1), and measurement of serum anti-GM-CSF IgG yielded results consistent with APAP. Serum autoantibody levels transiently decreased following WLL, but these returned to the initial levels at the time of remission and remained constant while the lung shadows changed (Figure 1). On the other hand, serum levels of Krebs von den Lungen-6 (KL-6), a sialylated glycoprotein expressed by alveolar type 2 cells and one of the biomarkers of alveolar proteinosis, well paralleled the changes in the lung shadows (Figure 1).
Clinical course of a 46-year-old man with autoimmune pulmonary alveolar proteinosis. IgG antibody against GM-CSF was measured by enzyme-linked immunosorbent assay using polyclonal reference IgG antibody purified from 10 patients with autoimmune pulmonary alveolar proteinosis. GM-CSF, granulocyte macrophage colony-stimulating factor; KL-6, Krebs von den Lungen-6
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F2
Five months after the last WLL, the patient was doing very well and lung shadows improved further (Figure 2). However, at 13 months after the last WLL, an apparent relapse was observed on CT scan (Figure 2). Apparent but modest improvement of lung shadows was observed 2 months later (Figure 2).