Fibrosing alveolitis in patients with neurofibromatosis.

Journal Article (Journal Article)

Fibrosing alveolitis, or interstitial pulmonary fibrosis, is a common manifestation of neurofibromatosis, and was observed in 7 of 70 patients with the disease. Though neurofibromatosis is congenital, fibrosing alveolitis does not appear until adulthood, and occurs in 20% of patients with the disease who are over 30 years old. Characteristic radiographic findings include linear, interstitial density, and large upper lobe bullae; this combination limits the differential diagnosis. Pathological examination demonstrates alveolar wall thickening progressing to fibrosis and lung destruction. Pulmonary function tests can show obstructive or restrictive lung disease.

Full Text

Duke Authors

Cited Authors

  • Webb, WR; Goodman, PC

Published Date

  • February 1, 1977

Published In

Volume / Issue

  • 122 / 2

Start / End Page

  • 289 - 293

PubMed ID

  • 402016

International Standard Serial Number (ISSN)

  • 0033-8419

Digital Object Identifier (DOI)

  • 10.1148/122.2.289


  • eng

Conference Location

  • United States