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Table 1 Etiology of diffuse cystic lung diseases

From: The etiology of diffuse cystic lung diseases: an analysis of 1010 consecutive cases in a LAM clinic

Causes Numbers (%)
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%)
  1. *This case of amyloidosis did not have Sjogren’s syndrome