Wang Si-Qin, Yang Zhi-Gang, Ma Xi-Tao
Department of Respiratory Medicine, Henan Provincial Hospital, Zhengzhou 450003, China.
Zhonghua Jie He He Hu Xi Za Zhi. 2008 Aug;31(8):591-3.
To investigate the clinical features of familial idiopathic pulmonary fibrosis (FIPF) and therefore to improve the recognition of the disease.
Clinical data of 5 patients with idiopathic pulmonary fibrosis belonging to 2 families were analyzed retrospectively.
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.
The clinical features of FIPF are similar to those of nonfamilial IPF. Asymptomatic FIPF can be identified early by lung HRCT.
探讨家族性特发性肺纤维化（FIPF）的临床特征，以提高对该病的认识。
回顾性分析2个家族中5例特发性肺纤维化患者的临床资料。
一个家族有3例患者，另一个家族有2例患者。5例FIPF患者首次诊断时的平均年龄为（55±12）岁。最常见的初始症状是咳嗽和进行性呼吸困难、双肺底吸气末捻发音和杵状指。高分辨率CT（HRCT）显示网状阴影和弥漫性蜂窝状改变。肺通气功能正常，但一氧化碳弥散量降低。1例经外科肺活检确诊为普通型间质性肺炎。
FIPF的临床特征与非家族性特发性肺纤维化相似。无症状的FIPF可通过肺部HRCT早期识别。
