title: [Familial idiopathic pulmonary fibrosis: case report and review of literature].
abstract: 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.
authors.affiliation: Department of Respiratory Medicine, Henan Provincial Hospital, Zhengzhou 450003, China.
authors.forename: Si-Qin
authors.initials: SQ
authors.lastname: Wang
authors.forename: Zhi-Gang
authors.initials: ZG
authors.lastname: Yang
authors.forename: Xi-Tao
authors.initials: XT
authors.lastname: Ma
mesh_terms: D000368:Aged; D001706:Biopsy; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed