[Seven cases of marked pulmonary fibrosis in the upper lobe]

Nihon Kokyuki Gakkai Zasshi. 1999 Feb;37(2):87-96.
[Article in Japanese]


We report on 7 patients with marked idiopathic pulmonary fibrosis in the upper lung lobes. The patients were generally characterized by the following pathological and clinical features: (1) marked subpleural pulmonary opacities and reticular shadows mainly in the upper lobes, with a progressive reduction in lung volume; (2) nonspecific fibrosis (subpleural, zonal, rather well-defined fibrosis with small cysts and honeycomb lesions) in 5 patients and interstitial pneumonia in 2, mainly in the upper lobes; and (3) a slender build, and a family history of recurrent spontaneous pneumothorax. Clinically, all 7 cases resembled idiopathic upper-lobe fibrosis as described by Amitani in 1992. Further clinical and experimental research will be required to delineate the characteristics of idiopathic fibrosis affecting primarily the upper lobes.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Cystic Fibrosis / pathology
  • Female
  • Humans
  • Lung / pathology*
  • Male
  • Middle Aged
  • Pneumothorax / complications
  • Pulmonary Fibrosis / pathology*