Table 2.

Characteristics of Affected Subjects

	Kindred
	A	B	C	D	E	F	G	H	I	Total
Number of affected	8	3	9	7	4	6	4	4	4	49
Age at diagnosis	45–72	76–87	57–75	68–77	66–75	45–78	65–73	49–63	58–82	64.8 (10.6)
Tobacco use	4	1	3	3	1	1	2	3	1	19 (38.8%)
Diagnosis
Definite	6		4			1	2	3	1	17 (34.7%)
Probable	2	2	2	3	2	1	2	1	1	16 (32.6%)
Possible			2	4	2	4			2	14 (28.6%)
Other ILD		1	1							2 (4.1%)
FVC, %	58–75	55–86		58–84	77–79	60	90–102	31–79	42–63	70.3 (16.9)
DlCO, %	45–82	33–58	19–85	34–89	33–85	53	34–94	73–93	33–55	54.1 (21.9)

Definition of abbreviations: Dl_CO = diffusing capacity of the lung for carbon monoxide; ILD = interstitial lung disease.

Data are presented as mean (SD) or number (percent). Pulmonary function tests were not available for all subjects.