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 DPLDb | 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