A Newfoundland cohort of familial and sporadic idiopathic pulmonary fibrosis patients: clinical and genetic features

Table 4 Idiopathic interstitial pneumonia (IIP) histologies among FPF and sporadic PF patients with a tissue confirmed diagnosis (surgical lung biopsy or autopsied lungs)

	All FPF patients (n = 77)	Sporadic PF patients (n = 50)
Number of patients with a tissue diagnosis (%)	33 (42.9 %)	21 (51 %)
Number of patients with IPF/UIP histology (%)	27 (81.8 %)	16 (76.2 %)
Number of patients with mixed UIP and NSIP histology patterns (%)	1^a	1
Number with IIP other than UIP (%):	3	2
NSIP	0	0
COP	0	0
AIP	0	0
RB-ILD	0	0
DIP	0	0
LIP Unclassifiable interstitial pneumonia	2 ^a	2
Total non-UIP histologic pattern	6 (18.2 %)	4 (19 %)

NSIP = non-specific interstitial pneumonia, COP = cryptogenic organizing pneumonia, AIP = acute interstitial pneumonia, RB-ILD = respiratory bronchiolitis-associated interstitial lung disease, DIP = desquamative interstitial pneumomia, LIP = lymphocytic interstitial pneumonia.
^a One biopsy showing a mix of UIP and NSIP and one biopsy showing unclassifiable interstitial pneumonia come from 2 members of the same family (R0892). The other 4 non-UIP biopsies from PF patients come from four different families.

ISSN: 1465-993X