Familial idiopathic pulmonary fibrosis. Evidence of lung inflammation in unaffected family members. Academic Article uri icon

Overview

MeSH

  • Adult
  • Aged
  • Biopsy
  • Female
  • Gallium Radioisotopes
  • Genes, Dominant
  • Humans
  • Inflammation
  • Lung
  • Macrophages
  • Male
  • Middle Aged
  • Neutrophils

MeSH Major

  • Pulmonary Alveoli
  • Pulmonary Fibrosis

abstract

  • We evaluated 17 clinically unaffected members of three families with an autosomal dominant form of idiopathic pulmonary fibrosis for evidence of alveolar inflammation. Each person in the study was examined by gallium-67 scanning for a general estimate of pulmonary inflammation, and by bronchoalveolar lavage for characterization of the types of recovered cells and their state of activation. Eight of the 17 subjects had evidence of alveolar inflammation on the lavage studies. Supporting data included increased numbers of neutrophils and activated macrophages that released one or more neutrophil chemoattractants, and growth factors for lung fibroblasts--findings similar to those observed in patients with overt idiopathic pulmonary fibrosis. Four of these eight also had a positive gallium scan; in all the other clinically unaffected subjects the scan was normal. During a follow-up of two to four years in seven of the eight subjects who had evidence of inflammation, no clinical evidence of pulmonary fibrosis has appeared. These results indicate that alveolar inflammation occurs in approximately half the clinically unaffected family members at risk of inheriting autosomal dominant idiopathic pulmonary fibrosis. Whether these persons with evidence of pulmonary inflammation but no fibrosis will proceed to have clinically evident pulmonary fibrosis is not yet known.

publication date

  • May 22, 1986

has subject area

  • Adult
  • Aged
  • Biopsy
  • Female
  • Gallium Radioisotopes
  • Genes, Dominant
  • Humans
  • Inflammation
  • Lung
  • Macrophages
  • Male
  • Middle Aged
  • Neutrophils
  • Pulmonary Alveoli
  • Pulmonary Fibrosis

Research

keywords

  • Case Reports
  • Journal Article

Identity

Language

  • eng

Digital Object Identifier (DOI)

  • 10.1056/NEJM198605223142103

PubMed ID

  • 3702942

Additional Document Info

start page

  • 1343

end page

  • 1347

volume

  • 314

number

  • 21