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

Electronic International Standard Serial Number (EISSN)

  • 1543-2165

Digital Object Identifier (DOI)

  • 10.5858/arpa.2011-0627-OAI


  • eng

Conference Location

  • United States