Familial Idiopathic Pulmonary Fibrosis

Peter B. Bitterman, Stephen I. Rennard, Brendan A. Keogh, Mark D. Wewers, Steven Adelberg, Ronald Crystal

Research output: Contribution to journalArticle

134 Citations (Scopus)

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 chemo-attractants, 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. (N Engl J Med 1986; 314:1343–7.), IDIOPATHIC pulmonary fibrosis is a chronic, progressive, usually fatal interstitial lung disorder characterized by inflammation and fibrosis of the alveolar walls.

Original languageEnglish
Pages (from-to)1343-1347
Number of pages5
JournalNew England Journal of Medicine
Volume314
Issue number21
DOIs
Publication statusPublished - 22 May 1986
Externally publishedYes

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Idiopathic Pulmonary Fibrosis
Pulmonary Fibrosis
Inflammation
Gallium
Pneumonia
Neutrophils
Fibrosis
Lung
Therapeutic Irrigation
Bronchoalveolar Lavage
Intercellular Signaling Peptides and Proteins
Macrophages

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Bitterman, P. B., Rennard, S. I., Keogh, B. A., Wewers, M. D., Adelberg, S., & Crystal, R. (1986). Familial Idiopathic Pulmonary Fibrosis. New England Journal of Medicine, 314(21), 1343-1347. https://doi.org/10.1056/NEJM198605223142103

Familial Idiopathic Pulmonary Fibrosis. / Bitterman, Peter B.; Rennard, Stephen I.; Keogh, Brendan A.; Wewers, Mark D.; Adelberg, Steven; Crystal, Ronald.

In: New England Journal of Medicine, Vol. 314, No. 21, 22.05.1986, p. 1343-1347.

Research output: Contribution to journalArticle

Bitterman, PB, Rennard, SI, Keogh, BA, Wewers, MD, Adelberg, S & Crystal, R 1986, 'Familial Idiopathic Pulmonary Fibrosis', New England Journal of Medicine, vol. 314, no. 21, pp. 1343-1347. https://doi.org/10.1056/NEJM198605223142103
Bitterman PB, Rennard SI, Keogh BA, Wewers MD, Adelberg S, Crystal R. Familial Idiopathic Pulmonary Fibrosis. New England Journal of Medicine. 1986 May 22;314(21):1343-1347. https://doi.org/10.1056/NEJM198605223142103
Bitterman, Peter B. ; Rennard, Stephen I. ; Keogh, Brendan A. ; Wewers, Mark D. ; Adelberg, Steven ; Crystal, Ronald. / Familial Idiopathic Pulmonary Fibrosis. In: New England Journal of Medicine. 1986 ; Vol. 314, No. 21. pp. 1343-1347.
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