Table 1 Etiology of diffuse cystic lung diseases

Lymphangioleiomyomatosis

711 (70.4%)

Birt–Hogg–Dubé syndrome

46 (4.6%)

Sjogren syndrome

38 (3.8%)

Light-chain deposition disease

2

Amyloidosis

2

Lymphocytic interstitial pneumonitis

1

Pulmonary Langerhans cell histiocytosis

14 (1.4%)

Tumor

3 (0.3%)

Castleman’s disease

2 (0.2%)

Antineutrophil cytoplasmic antibody-associated vasculitis

2 (0.2%)

Systemic lupus erythematosus

1 (0.1%)

Marfan syndrome

1 (0.1%)

Amyloidosis*

1 (0.1%)

Congenital cystic adenomatoid malformation of the lung

1 (0.1%)

Pleuroparenchymal fibroelastosis

1 (0.1%)

Undiagnosed diffuse cystic lung diseases

189 (18.7%)

Total

1010 (100%)