HOME > Pulmonary Alveolar Proteinosis > Diagnostic Criteria for autoimmune PAP

Pulmonary Alveolar Proteinosis


Link
Measurement of GM-CSF autoantibody

Pulmonary Alveolar Proteinosis

Diagnostic Criteria for autoimmune PAP

Diagnostic criteria

The diagnosis of PAP is based on the following criteria.
The findings of the patients meet all of the three below.

  1. Radiographic findings on HRCT characteristic of autoimmune PAP include a diffuse or patchy “geographic” pattern of ground glass opacification superimposed on interlobular septal thickening in multiple lobes.
  2. Pathological/Cytological Findings:
    a. Characteristic pathological features include intraalveolar, eosinophilic, periodic acid-Schiff positive material in the biopsy specimen.
    b. Macroscopically, BALF is turbid. Microscopically, there is granular, eosinophilic, amorphous, or periodic acid-Schiff positive material and foamy macrophages.
  3. The serum is positive for GM-CSF autoantibodies.

Notes:
1. Of 223 cases with autoimmune PAP in Japan, 131 cases were diagnosed by CT and BALF findings, 76 cases were diagnosed by CT, BALF, and pathological features in TBLB specimens. 16 cases were diagnosed by CT and pathological findings in VATS samples(ref.2)

PAGE TOP