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

Table 2 Demographic characteristics among different causes of DCLD

	Numbers	Age (years)	Female (%)	Smoking history	Family history
LAM	711	38.2 ± 10.8	711(100.0%)	1.1%	1.1%
BHD	46	47.3 ± 10.4	42 (91.3%)	6.5%	80.4%
SS	38	49.7 ± 10.4	38 (100.0%)	2.6%	5.3%
PLCH	14	32.8 ± 12.4	6 (42.9%)	92.9%	0
Tumor	3	41–50	3 (100.0%)	0	0
CD	2	34–46	1 (50%)	50%	0
AVA	2	37–51	2 (100%)	0	0
SLE	1	45	1 (100%)	0	0
MFS	1	57	0	0	100.0%
AMY	1	32	1 (100.0%)	0	0
CCAM	1	28	1 (100.0%)	0	0
PPFE	1	37	1 (100.0%)	0	0

AMY amyloidosis, AVA antineutrophil cytoplasmic antibody-associated vasculitis, BHD Birt–Hogg–Dubé syndrome, CCAM congenital cystic adenomatoid malformation of the lung, CD Castleman disease, DCLD diffuse cystic lung disease, LAM lymphangioleiomyomatosis, MFS Marfan syndrome, PPFE pleuroparenchymal fibroelastosis, PLCH pulmonary Langerhans cell histiocytosis, SS Sjogren's syndrome

ISSN: 1750-1172