Inherited forms of interstitial lung disease
Rosenberg, D.M.
Clinics in Chest Medicine 3(3): 635-641
1982
ISSN/ISBN: 0272-5231 PMID: 6751674 Document Number: 5424
Inherited diseases affecting the interstitium of the lung are few in number. The significance of the subject, however, may actually be inversely proportional to the frequency of these disorders. One reason for this potential importance rests on the fact that genetic disorders often can be utilized as models upon which to establish pathophysiologic mechanisms. It is hoped that once the mechanisms for inherited forms of interstitial lung disease have been elucidated, they could subsequently be applied to the numerous acquired diseases associated with interstitial lung involvement. In addition, while hereditary forms of interstitial lung disease are infrequent, it is conceivable that genetic mechanisms are responsible for the fibrosis seen in all disorders involving the lung interstitium. Along these lines, it has been postulated that a gene tic predisposition, in the form of a fibrotic gene exists and under certain circumstances (that is, environmental exposure or acquired disease) regulation over the gene is impaired and interstitial involvement follows. This overview of the hereditary forms of interstitial lung disease will begin by reviewing the fibrotic gene theory, then concentrate on specific genetically based interstitial lung disorders.
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