Idiopathic pulmonary fibrosis: is it a familial disease?
JS Allam, AH Limper - Current opinion in pulmonary medicine, 2006 - journals.lww.com
JS Allam, AH Limper
Current opinion in pulmonary medicine, 2006•journals.lww.comThe pathogenesis of both idiopathic pulmonary fibrosis and familial idiopathic pulmonary
fibrosis remains unclear. A consistent genetic basis has not yet been demonstrated in all
cases. Other factors, including variable gene expression, co-carriage of other modifying
genes and environmental stimuli, particularly cigarette smoke, significantly contribute to
disease expression.
fibrosis remains unclear. A consistent genetic basis has not yet been demonstrated in all
cases. Other factors, including variable gene expression, co-carriage of other modifying
genes and environmental stimuli, particularly cigarette smoke, significantly contribute to
disease expression.
Summary
The pathogenesis of both idiopathic pulmonary fibrosis and familial idiopathic pulmonary fibrosis remains unclear. A consistent genetic basis has not yet been demonstrated in all cases. Other factors, including variable gene expression, co-carriage of other modifying genes and environmental stimuli, particularly cigarette smoke, significantly contribute to disease expression.
Lippincott Williams & Wilkins