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Table 2 Prevalence of familial DPLD cases vs. sporadic forms

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

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 DPLDb 32
Total 79 287  
  1. 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