Kono Masato, Nakamura Yutaro, Enomoto Noriyuki, Hashimoto Dai, Fujisawa Tomoyuki, Inui Naoki, Maekawa Masato, Suda Takafumi, Colby Thomas V, Chida Kingo
Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan; Department of Laboratory Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
PLoS One. 2014 Apr 15;9(4):e94775. doi: 10.1371/journal.pone.0094775. eCollection 2014.
The aim of this study was to evaluate the cumulative incidence and the predictive factors for collagen vascular disease (CVD) in patients initially diagnosed with idiopathic pulmonary fibrosis (IPF), and to examine the features of patients who then developed CVD.
This was a retrospective review of 111 consecutive patients with IPF diagnosed at our institution. None of the patients fulfilled any of the CVD criteria from the American College of Rheumatology (ACR) within 6 months or more after the diagnosis of IPF.
Ten patients (9.0%) developed CVD during the follow-up period: four had rheumatoid arthritis (RA); four had microscopic polyangiitis (MPA); one had systemic sclerosis (SSc); and one had SSc and Sjogren's syndrome (SjS). The mean time until CVD diagnosis was 3.9 years. The cumulative incidences of CVD at 1, 5, and 10 years were 0.91%, 9.85%, and 15.5%, respectively. Patients who developed CVD were significantly younger, more likely to be women and had a better prognosis than those with IPF. Cox proportional hazards regression analysis showed that female sex and the presence of lymphoid aggregates with germinal centers were significantly associated with the occurrence of CVD in patients initially diagnosed with IPF.
CVD is an important underlying condition in IPF, and shows better prognosis. The possibility of the development of CVD should remain a consideration in the follow-up of IPF.
本研究旨在评估初诊为特发性肺纤维化（IPF）患者的胶原血管病（CVD）累积发病率及预测因素，并研究随后发生CVD患者的特征。
这是一项对在我院确诊的111例连续性IPF患者的回顾性研究。在IPF诊断后6个月或更长时间内，无患者符合美国风湿病学会（ACR）的任何CVD标准。
10例患者（9.0%）在随访期间发生CVD：4例患有类风湿关节炎（RA）；4例患有显微镜下多血管炎（MPA）；1例患有系统性硬化症（SSc）；1例患有SSc和干燥综合征（SjS）。至CVD诊断的平均时间为3.9年。1年、5年和10年时CVD的累积发病率分别为0.91%、9.85%和15.5%。发生CVD的患者比IPF患者显著更年轻、更可能为女性且预后更好。Cox比例风险回归分析显示，女性性别和存在生发中心的淋巴样聚集与初诊为IPF患者发生CVD显著相关。
CVD是IPF的一种重要基础疾病，且预后较好。在IPF随访中应始终考虑发生CVD的可能性。
