|
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
