Chronic interstitial pneumonia in young patients undergoing lung transplantation or autopsy: clinico-radiologic-pathologic observations from a single institution

Purpose

To retrospectively evaluate high-resolution computed tomography (HRCT) findings and clinical diagnoses of chronic interstitial pneumonia (IP) with a poor prognosis in young patients (≤50 years).

Materials and methods

HRCT images of 8 men and 7 women (mean age 34.8 years) obtained before lung transplantation or autopsy were reviewed. After reviewing whole lung specimens and pathologic diagnoses, all patients were clinically diagnosed according to the 2010 idiopathic pulmonary fibrosis/usual interstitial pneumonia (IPF/UIP) consensus statement.

Results

HRCT images revealed intralobular reticular opacity, air cysts, ground glass opacity, traction bronchiectasis, and interlobular septal thickening. Intralobular reticular opacity was the most extensive finding. Abnormal findings existed predominantly in both the peripheral and lower lung zones in only 1 patient. Classifications of HRCT patterns were “UIP” (n = 2), “inconsistent with UIP” (n = 11), and “indeterminate UIP” (n = 2). Multidisciplinary diagnoses were “IPF/UIP” (n = 1), “possible IPF/UIP” (n = 1), “IP with connective tissue disease” (n = 7), “fibrotic nonspecific IP” (n = 1), and “unclassified IP” (n = 5).

Conclusion

The most extensive HRCT finding was intralobular reticular opacity. Most HRCT images differed from typical IPF/UIP, and IPF/UIP was uncommon in young patients with chronic IP with a poor prognosis.