Inflammation and Fibrosis in patients with Interstitial Pneumonia

特发性间质性肺炎(IIP)包括一系列临床病理疾病，如特发性肺纤维化(IPF)，其组织学诊断为普通型间质性肺炎(DIP)、非特异性间质性肺炎(NSIP)、脱屑性间质性肺炎(DIP)、呼吸性毛细支气管炎相关性间质性肺疾病(RB-ILD)、闭塞性细支气管炎组织性肺炎(BOOP)、急性间质性肺炎(AIP)和淋巴管炎性间质性肺炎(LIP)。间质性肺炎伴胶原性血管疾病(IP-CVD)是与IIP相鉴别的候选疾病。在IIPS中，DIP-组织学与预后不良有关，而在DIP-CVD中，组织学特征与预后无关。为了探讨特发性病例与脑血管病(CVD)的临床病理特征的差异，比较了IPP/UIP和UIP-CVD的临床病理特征。UIP-CVD患者BAL液淋巴细胞增多，而IPF/UIP患者BAL液淋巴细胞增加不明显。这种差异可能与纤维化程度的不同有关；UIP-CVD患者的纤维化程度比IPF/UIP患者轻。BAL液淋巴细胞增多症是否对肺纤维化的发展起到调节作用还有待研究。在特发性病例中，肺纤维化的性质可以在DIP组织学和NSIP组织学之间发现差异。还将审查是否可以在与心血管疾病有关的病例中表现出类似的差异。

Idiopathic interatitial pneumonia (IIP) comprises a heterogeneous spectrum of clinicopathological disease entities such as idiopathic pulmonary fibrosia (IPF) the histology of which is diagnosed as usual interstitial pneumonia (DIP), nonspecific interstitial pneumonia (NSIP), desquamative interstitial pneumonia (DIP), respiratory bronchiolitis associated interstitial lung diseases (RB-ILD), bronchiolitis obliterans organizing pneumonia (BOOP), acute interstitial pneumonia (AIP), andlysiphocytic interstitial pneumonia (LIP). Interstitial pneumonia associated with collagen vascular diseases (IP-CVD) are candidate for differentiation to IIP. Among the IIPs, the DIP-histology relates to an unfavorable prognosis, however, in the cases of DIP-CVD, histological features are not likely to relate to prognosis. In order to investigate a difference between idiopathic cases and CVD with UIP-histology, or with NSIP-histology,clinicopathological features were compared between IPP/UIP and UIP-CVD.An increase in BAL fluid lymphocytes was found in patients with UIP-CVD, but not in patients with IPF/UIP. This differences may relate to a difference in the degree of fibrosis ; those in patients with UIP-CVD were milder than those in patients with IPF/UIP. It remains to be examined whether a BAL fluid lymphocytosis plays a role of regulating a development of pulmonary fibrosis. Differences in the nature of pulmonary fibrosis can be found between the DIP-histology and the NSIP-histology in the idioapthic cases. It also will be examined whether a similar difference can be shown in the cases associated with CVD.