[Familial idiopathic pulmonary fibrosis: case report and review of literature].

OBJECTIVE

To investigate the clinical features of familial idiopathic pulmonary fibrosis (FIPF) and therefore to improve the recognition of the disease.

METHODS

Clinical data of 5 patients with idiopathic pulmonary fibrosis belonging to 2 families were analyzed retrospectively.

RESULTS

There were 3 patients in one family and 2 in another family. The average age at first diagnosis of the 5 patients with FIPF were (55 +/- 12) years. The most common initial symptoms were cough and progressive dyspnea, bibasilar end-inspiratory Velcro and clubbed fingers. HRCT revealed reticular opacities and diffuse honeycombing. Pulmonary ventilatory function was normal, but the diffusing capacity for carbon monoxide was reduced. One case was confirmed to have usual interstitial pneumonia by surgical lung biopsy.

CONCLUSIONS

The clinical features of FIPF are similar to those of nonfamilial IPF. Asymptomatic FIPF can be identified early by lung HRCT.