Department of Respiratory Medicine, Kobe City Medical Center General Hospital, Kobe, Japan.
Respiration. 2012;83(1):20-7. doi: 10.1159/000329893. Epub 2011 Sep 6.
Relatively little is known about acute exacerbation (AE) of interstitial pneumonia associated with collagen vascular diseases (CVD-IPs).
This study was aimed at clarifying clinical characteristics and outcome in AE of CVD-IPs, compared with those of idiopathic interstitial pneumonias (IIPs).
We retrospectively reviewed 112 admission cases with suspected AE of CVD-IPs or IIPs during 2003-2009. IIPs were diagnosed with idiopathic pulmonary fibrosis (IPF) or non-IPF, mostly based on radiologic findings. Of these, 15 AEs of CVD-IPs (6 rheumatoid arthritis, 6 dermatomyositis and 3 systemic sclerosis) and 47 AEs of IIPs (13 IPF and 34 non-IPF) were included.
The clinical characteristics in AE of CVD-IPs were similar to those of IIPs, except for younger age (63.3 ± 6.8 vs. 73.8 ± 9.1 years; p = 0.0001) and higher PaO(2)/FiO(2) at the onset of AE (205 ± 81.2 vs. 145 ± 53.8 mm Hg; p = 0.002) in the former. Dermatomyositis-related interstitial pneumonia (IP) showed a relatively indolent onset and was often associated with worsening control of the underlying disease, whereas AE of other CVD-IPs resembled that of IIPs. 90-day mortality of 33% in AE of CVD-IPs was similar to that of IIPs (44%; p = 0.44) or non-IPF (34%; p = 0.94), but was significantly better than that of IPF (69%; p = 0.04).
Clinical features and outcome in AE of CVD-IPs were similar, if not identical, to those of IIPs, having a significant impact on the clinical course. AE of advanced IPF with typical radiologic features seems to have higher mortality compared with other forms of IP.
人们对与胶原血管疾病相关的间质性肺炎急性加重（AE）了解甚少。
本研究旨在阐明与特发性间质性肺炎（IIP）相比，胶原血管疾病相关间质性肺炎 AE 的临床特征和结局。
我们回顾性分析了 2003-2009 年间疑似胶原血管疾病相关间质性肺炎或特发性间质性肺炎 AE 的 112 例住院病例。IIP 主要基于影像学表现诊断为特发性肺纤维化（IPF）或非 IPF。其中，15 例胶原血管疾病相关间质性肺炎 AE（6 例类风湿关节炎、6 例皮肌炎和 3 例系统性硬化症）和 47 例特发性间质性肺炎 AE（13 例 IPF 和 34 例非 IPF）纳入研究。
胶原血管疾病相关间质性肺炎 AE 的临床特征与 IIP 相似，但胶原血管疾病相关间质性肺炎 AE 患者的年龄较小（63.3±6.8 岁比 73.8±9.1 岁；p=0.0001），AE 发病时的 PaO2/FiO2 较高（205±81.2 比 145±53.8 mm Hg；p=0.002）。皮肌炎相关间质性肺炎（IP）起病相对较隐匿，常与基础疾病控制恶化相关，而其他胶原血管疾病相关间质性肺炎 AE 与 IIP 相似。胶原血管疾病相关间质性肺炎 AE 的 90 天死亡率为 33%，与 IIP（44%；p=0.44）或非 IPF（34%；p=0.94）相似，但显著低于 IPF（69%；p=0.04）。
胶原血管疾病相关间质性肺炎 AE 的临床特征和结局与 IIP 相似（即使不完全相同），对临床病程有显著影响。与其他形式的 IP 相比，具有典型影像学特征的晚期 IPF 的 AE 似乎死亡率更高。
