Abstract：
BACKGROUND: Patients with idiopathic interstitial pneumonia (IIP) have a favourable prognosis when they have interstitial pneumonia with autoimmune features (IPAF). However, precise IPAF-related findings from high-resolution computed tomography (HRCT) and lung histopathological specimens and the treatment response have not been fully determined. Therefore, this study was conducted to evaluate the relationship between findings on HRCT or lung histopathological specimens and the progression of interstitial pneumonia in patients with IPAF.
METHODS: This multicentre cohort study prospectively enrolled consecutive patients with IIP. At the diagnosis of IIP, we systematically evaluated 74 features suggestive of connective tissue diseases and followed them up. HRCT, lung specimens, serum antibodies, and the clinical course were also evaluated.
RESULTS: Among 222 patients with IIP, 26 (11.7%) fulfilled the IPAF criteria. During a median observation period of 36 months, patients with IPAF showed better survival than those without IPAF (p = 0.034). While histopathological findings were not related to IPAF, nonspecific interstitial pneumonia (NSIP) with organizing pneumonia (OP) overlap was the most prevalent HRCT pattern (p < 0.001) and the consolidation opacity was the most common radiological finding in IPAF (p = 0.017). Furthermore, in patients with IPAF, the diagnosis of COP or NSIP with OP overlap was associated with a higher increase in %FVC in 1 year than in those with idiopathic pulmonary fibrosis, NSIP, or unclassifiable IIP (p = 0.002).
CONCLUSIONS: This study shows the presence of consolidation opacity on HRCT and the diagnosis of COP or NSIP with OP overlap are associated with IPAF and its favourable treatment response in patients with IPAF.
METHODS: This multicentre cohort study prospectively enrolled consecutive patients with IIP. At the diagnosis of IIP, we systematically evaluated 74 features suggestive of connective tissue diseases and followed them up. HRCT, lung specimens, serum antibodies, and the clinical course were also evaluated.
RESULTS: Among 222 patients with IIP, 26 (11.7%) fulfilled the IPAF criteria. During a median observation period of 36 months, patients with IPAF showed better survival than those without IPAF (p = 0.034). While histopathological findings were not related to IPAF, nonspecific interstitial pneumonia (NSIP) with organizing pneumonia (OP) overlap was the most prevalent HRCT pattern (p < 0.001) and the consolidation opacity was the most common radiological finding in IPAF (p = 0.017). Furthermore, in patients with IPAF, the diagnosis of COP or NSIP with OP overlap was associated with a higher increase in %FVC in 1 year than in those with idiopathic pulmonary fibrosis, NSIP, or unclassifiable IIP (p = 0.002).
CONCLUSIONS: This study shows the presence of consolidation opacity on HRCT and the diagnosis of COP or NSIP with OP overlap are associated with IPAF and its favourable treatment response in patients with IPAF.
摘要：
背景：特发性间质性肺炎（IIP）患者如果患有具有自身免疫特征的间质性肺炎（IPAF），预后良好。然而,高分辨率计算机断层扫描(HRCT)和肺组织病理学标本的IPAF相关结果和治疗反应尚未完全确定.因此,本研究旨在评估HRCT或肺组织病理学标本的发现与IPAF患者间质性肺炎进展之间的关系.
方法：这项多中心队列研究前瞻性招募了连续的IIP患者。在IIP的诊断中，我们系统评估了提示结缔组织疾病的74项特征并进行了随访.HRCT,肺标本,血清抗体，并对临床病程进行了评估。
结果：在222例IIP患者中，26（11.7%）符合IPAF标准。在36个月的中位观察期内，IPAF患者的生存率优于无IPAF患者(p=0.034).虽然组织病理学发现与IPAF无关，非特异性间质性肺炎(NSIP)与机化性肺炎(OP)重叠是最常见的HRCT模式(p<0.001),巩固性不透明是IPAF中最常见的放射学表现(p=0.017).此外，在IPAF患者中，与特发性肺纤维化患者相比，COP或NSIP与OP重叠的诊断与1年内％FVC的增加有关，NSIP,或不可分类的IIP(p=0.002)。
结论：本研究显示HRCT上实变不透明的存在以及COP或NSIP与OP重叠的诊断与IPAF及其在IPAF患者中的良好治疗反应相关。
方法：这项多中心队列研究前瞻性招募了连续的IIP患者。在IIP的诊断中，我们系统评估了提示结缔组织疾病的74项特征并进行了随访.HRCT,肺标本,血清抗体，并对临床病程进行了评估。
结果：在222例IIP患者中，26（11.7%）符合IPAF标准。在36个月的中位观察期内，IPAF患者的生存率优于无IPAF患者(p=0.034).虽然组织病理学发现与IPAF无关，非特异性间质性肺炎(NSIP)与机化性肺炎(OP)重叠是最常见的HRCT模式(p<0.001),巩固性不透明是IPAF中最常见的放射学表现(p=0.017).此外，在IPAF患者中，与特发性肺纤维化患者相比，COP或NSIP与OP重叠的诊断与1年内％FVC的增加有关，NSIP,或不可分类的IIP(p=0.002)。
结论：本研究显示HRCT上实变不透明的存在以及COP或NSIP与OP重叠的诊断与IPAF及其在IPAF患者中的良好治疗反应相关。
