Autoimmune Lung Center and Interstitial Lung Disease Program, National Jewish Health, Denver, CO 80206, USA.
Respir Med. 2013 Jun;107(6):883-9. doi: 10.1016/j.rmed.2013.02.019. Epub 2013 Apr 10.
Fibrosis or inflammation of the bronchioles is a well-known manifestation of connective tissue disease (CTD). However, the natural history of CTD-related bronchiolitis is largely unknown.
We analyzed consecutive patients evaluated at National Jewish Health (Denver, CO) from 1998 to 2008 with CTD and surgical lung biopsy-confirmed bronchiolitis. Linear mixed effects models were used to estimate the longitudinal postbronchodilator FEV1 %predicted (%pred) course and differences between subjects with or without constrictive bronchiolitis (CB).
Of 28 subjects with a mean age of 53 ± 9 years, fourteen (50%) had CB. The most common CTD diagnosis was rheumatoid arthritis (n = 14; 50%). There were no significant differences in demographics, smoking status, underlying CTD diagnoses, 6-min walk distance, dyspnea score or drug therapy between subjects with CB and those with cellular bronchiolitis. Three subjects with CB (11%) and four with cellular bronchiolitis (14%) died. Compared with subjects with CB, those with cellular bronchiolitis had higher mean FEV1 %pred at all times. There were no significant differences in FEV1 %pred slope within- or between-groups (CB vs. cellular bronchiolitis) preceding surgical lung biopsy or afterward.
Subjects with CTD-related CB had lower FEV1 %pred values than those with CTD-related cellular bronchiolitis at all time points, but FEV1 %pred remained stable over time in both groups regardless of therapy received.
细支气管的纤维化或炎症是结缔组织病（CTD）的一种众所周知的表现。然而，CTD 相关细支气管炎的自然病史在很大程度上尚不清楚。
我们分析了 1998 年至 2008 年在丹佛国家犹太健康中心（科罗拉多州）接受评估的连续患者，这些患者患有 CTD 和手术肺活检证实的细支气管炎。线性混合效应模型用于估计支气管扩张后支气管舒张剂后的 FEV1%预测值（%pred）的纵向过程以及存在或不存在收缩性细支气管炎（CB）的受试者之间的差异。
在 28 名平均年龄为 53±9 岁的受试者中，有 14 名（50%）患有 CB。最常见的 CTD 诊断是类风湿关节炎（n=14；50%）。在 CB 患者和细胞性细支气管炎患者之间，在人口统计学、吸烟状况、潜在 CTD 诊断、6 分钟步行距离、呼吸困难评分或药物治疗方面没有显着差异。三名患有 CB（11%）和四名患有细胞性细支气管炎（14%）的患者死亡。与 CB 患者相比，细胞性细支气管炎患者在所有时间的平均 FEV1%pred 都更高。在手术肺活检之前或之后，在组内和组间（CB 与细胞性细支气管炎），FEV1%pred 斜率均无显着差异。
在所有时间点，患有 CTD 相关 CB 的受试者的 FEV1%pred 值均低于患有 CTD 相关细胞性细支气管炎的受试者，但无论接受何种治疗，两组的 FEV1%pred 值均保持稳定。
