Familial idiopathic pulmonary fibrosis: serial high-resolution computed tomography findings in 9 patients

J Comput Assist Tomogr. 2004 Jul-Aug;28(4):443-8. doi: 10.1097/00004728-200407000-00002.


Objective: The purpose of this study was to assess the high-resolution computed tomography (CT) findings of familial idiopathic pulmonary fibrosis (IPF).

Methods: High-resolution CT scans obtained in 9 consecutive patients with biopsy-proven familial IPF were reviewed. The presence, distribution, and extent of various CT findings were evaluated.

Results: All 9 patients had ground-glass opacities, intralobular reticular opacities, and irregular thickening of the interlobular septa; 7 (78%) had traction bronchiectasis, 7 (78%) had small foci of consolidation; and 3 (33%) had honeycombing. The abnormalities involved mainly the lower lung zones in 6 patients and the upper lung zones in 2 patients and had no zonal predominance in 1 patient. Follow-up CT showed increased extent of disease in 8 patients.

Conclusions: The high-resolution CT findings of familial IPF resemble those of nonfamilial IPF. Familial IPF has a lower prevalence of honeycombing and a lower prevalence of predominant lower lung zone distribution than nonfamilial IPF, however.

MeSH terms

  • Adult
  • Aged
  • Anti-Inflammatory Agents / therapeutic use
  • Bronchiectasis / diagnostic imaging
  • Cyclophosphamide / therapeutic use
  • Female
  • Follow-Up Studies
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Lung / diagnostic imaging
  • Male
  • Methylprednisolone / therapeutic use
  • Middle Aged
  • Pulmonary Fibrosis / diagnostic imaging
  • Pulmonary Fibrosis / genetics*
  • Statistics, Nonparametric
  • Survival Rate
  • Tomography, X-Ray Computed / methods*
  • Treatment Outcome


  • Anti-Inflammatory Agents
  • Glucocorticoids
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Methylprednisolone