A Familial History of Pulmonary Fibrosis in Patients with Chronic Hypersensitivity Pneumonitis

作者：Okamoto Tsukasa; Miyazaki Yasunari^*; Tomita Makoto; Tamaoka Meiyo; Inase Naohiko

来源：Respiration, 2013, 85(5): 384-390.

DOI：10.1159/000338123

• Familial idiopathic pulmonary fibrosis
• Genetic susceptibility
• Hypersensitivity pneumonitis

Background: Hypersensitivity pneumonitis (HP) is an immunologically mediated lung disease induced by the inhalation of a variety of antigens. Patients with chronic HP often have a family history of pulmonary fibrosis. This strongly suggests that both genetic and environmental factors play an important role in the pathogenesis of chronic HP. Objectives: We aimed to investigate the epidemiology and clinical features of chronic HP patients with a family history of pulmonary fibrosis. Methods: We retrospectively reviewed the clinical information of 114 cases diagnosed with chronic HP with insidious onset between 1992 and 2009. Results: Twenty cases (17.5%) were identified as having a family history of pulmonary fibrosis. All of these patients had lived apart from their afflicted relatives for at least several decades. The familial cases were younger than the nonfamilial cases at onset (57.5 +/- 9.6 vs. 64.0 +/- 7.0 years old, p = 0.008). The predicted vital capacity percentage and partial pressure of oxygen in arterial blood gas were significantly higher in the familial cases. There were no differences between the 2 groups in gender, smoking history, bronchoalveolar lavage fluid profile, radiologic findings or other clinical features. Conclusions: We found a familial clustering in patients with chronic HP. Various factors including genetic susceptibility to pulmonary fibrosis and environmental factors may contribute to the development of familial chronic HP.

• 出版日期2013