Familial idiopathic interstitial pneumonia: histopathology and survival in 30 patients.
Journal Article (Journal Article)
CONTEXT: Familial idiopathic interstitial pneumonia (F-IIP) describes the unexplained occurrence of diffuse parenchymal lung disease in related individuals. Prevailing wisdom suggests that the histopathology of F-IIP is indistinguishable from that of idiopathic pulmonary fibrosis, namely, usual interstitial pneumonia (UIP). OBJECTIVE: To define the histopathology of F-IIP in lung tissue samples. DESIGN: Tissue sections from 30 patients with F-IIP, enrolled in a national research program, were evaluated by 3 pulmonary pathologists using 15 predefined histopathologic features. Each feature was recorded independently before a final diagnosis was chosen from a limited list dichotomized between UIP or "not UIP." These 2 groups were then compared to survival. RESULTS: The consensus diagnosis for the F-IIP cohort was an unclassifiable parenchymal fibrosis (60%), with a high incidence of histopathologic honeycombing, fibroblast foci, and smooth muscle in fibrosis. Usual interstitial pneumonia, strictly defined, was identified in less than half of the F-IIP cases (range, 23%-50%). Interobserver agreement was fair (κ = 0.37) for 2 observers for the overall diagnosis of UIP. Findings unexpected in UIP were prevalent. The survival for the entire F-IIP cohort was poor, with an estimated mortality of 93% and a median age at death of 60.9 years. Subjects with UIP had a shorter survival and younger age at death. CONCLUSIONS: Pulmonary fibrosis was the dominant histopathology identified in our patients, but diagnostic features of UIP were seen in less than 50% of the samples. Overall survival was poor, with mortality accelerated apparently by the presence of a UIP pattern of disease.
Full Text
Duke Authors
Cited Authors
- Leslie, KO; Cool, CD; Sporn, TA; Curran-Everett, D; Steele, MP; Brown, KK; Wahidi, MM; Schwartz, DA
Published Date
- November 2012
Published In
Volume / Issue
- 136 / 11
Start / End Page
- 1366 - 1376
PubMed ID
- 23106582
Pubmed Central ID
- 23106582
Electronic International Standard Serial Number (EISSN)
- 1543-2165
Digital Object Identifier (DOI)
- 10.5858/arpa.2011-0627-OAI
Language
- eng
Conference Location
- United States