Clinical characteristics of patients with familial idiopathic pulmonary fibrosis (f-IPF)

BMC Pulmonary Medicine

Table 2 Prevalence of familial DPLD cases vs. sporadic forms

Diagnosis	Index patients and relatives with IIP (n)	Comparator group (sporadic, n)	Percentage of familial cases in relation to sporadic and familial forms (%)^a
IPF	44	147	23
NSIP	2	17	10.5
unclassifiable IIP	1	69	1.4
COP	0	42	–
other IIP	0	12	–
All IIP	47	287	14.1
other DPLD^b	32	–	–
Total	79	287

Abbreviations: With regard to COP and other IIPs, it was not possible to make a statement about frequency, due to the low prevalence. ^a relative to the respective comparator group. Percentages refer to the respective total number (familial and sporadic patients); ^b DPLD diffuse parenchymal lung disease, IPF idiopathic pulmonary fibrosis, NSIP non-specific interstitial pneumonia, COP cryptogenic organizing pneumonia, unclassifiable IIP unclassifiable idiopathic interstitial pneumonia, other IIP = bronchiolitis with associated diffuse parenchymal lung disease, desquamative interstitial pneumonia, acute interstitial pneumonia, lymphoid interstitial pneumonia

ISSN: 1471-2466