背景:Th/To自身抗体可能与评估间质性肺病(ILD)患者有关,因为系统性硬化症(SSc)的临床诊断可能不明显。该研究的目的是描述一组Th/To自身抗体阳性的ILD患者的肺功能表现和演变。
方法:本方案纳入抗Th/To自身抗体阳性的ILD患者。登记基线临床特征,并进行生存分析以确定与较差生存率相关的危险因素.
结果:包括52例抗Th/To自身抗体阳性的ILD患者。只有21%的患者符合ACR/EULAR2013系统性硬化症分类标准,63.4%符合IPAFATS/ERS2015标准。25%的患者在随访期间死亡。呼吸衰竭是死亡的主要原因。29名患者(56%)对其他标志性SSc自身抗体呈阳性。最常见的HRCT模式是非特异性间质性肺炎(NISP)。生存率与收缩期肺动脉压(sPAP)密切相关,男性和HRCT纤维化程度;此外,与仅抗Th/To阳性的患者相比,其他具有标志的SSc自身抗体阳性的患者的生存率更差.76%的人表现为纤维化进行性肺病,FVC的绝对下降至少5%。
结论:只有一小部分的ILD患者Th/To符合被归类为SSc的标准;然而,最符合IPAF标准。高比例的患者表现为进行性纤维化肺病。生存率与sPAP有关,肺部疾病的程度,以及其他标志性SSc自身抗体的存在。
Th/To autoantibody may be relevant in evaluating patients with interstitial lung disease (ILD) because the clinical diagnosis of systemic sclerosis (SSc) may not be evident. The study\'s objective was to describe manifestations and evolution of pulmonary function in a cohort of ILD patients positive for Th/To autoantibodies.
ILD patients positive for anti-Th/To autoantibody were enrolled in this protocol. Baseline clinical features were registered, and survival analysis was performed to identify risk factors associated with worse survival.
Fifty-two patients positive for anti-Th/To autoantibodies with ILD were included. Only 21% of the patients fulfilled the ACR/EULAR 2013 systemic sclerosis classification criteria, and 63.4% fulfilled the IPAF ATS/ERS 2015 criteria. Twenty-five percent of the patients died during follow-up. Respiratory failure was the principal cause of death. Twenty-nine patients (56%) were positive for other hallmark SSc autoantibodies. The most frequent HRCT pattern was nonspecific interstitial pneumonia (NISP). Survival was strongly associated to the systolic pulmonary arterial pressure (sPAP), male sex and the extent of fibrosis in HRCT; besides, patients positive for other hallmark SSc autoantibodies had worse survival compared to those positive only to anti-Th/To. Seventy-six percent of them behaved as fibrotic progressive pulmonary disease, with an absolute decline of the FVC of at least 5%.
Only a small proportion of ILD patients positive for Th/To meet the criteria to be classified as SSc; however, most met criteria for IPAF. A high proportion of patients behave as progressive fibrotic pulmonary disease. Survival is associated with sPAP, the extent of lung disease, and the presence of other hallmark SSc autoantibodies.