Fibrosing alveolitis in patients with neurofibromatosis

Radiology. 1977 Feb;122(2):289-93. doi: 10.1148/122.2.289.


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.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Blister / pathology
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Male
  • Middle Aged
  • Neurofibromatosis 1 / complications*
  • Pulmonary Alveoli / pathology
  • Pulmonary Fibrosis / diagnostic imaging
  • Pulmonary Fibrosis / etiology*
  • Pulmonary Fibrosis / pathology
  • Radiography