Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Calzada de Tlalpan 4502, Tlalpan, Sección XVI, 14080 Ciudad de México, Mexico.
Experimental Medicine Research Unit, Facultad de Medicina, Universidad Nacional Autónoma de México, Ciudad de México, Mexico.
Med Clin (Barc). 2024 Apr 26;162(8):378-384. doi: 10.1016/j.medcli.2023.11.023. Epub 2024 Jan 29.
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.
抗 Th/To 自身抗体可能与评估间质性肺疾病（ILD）患者有关，因为系统性硬化症（SSc）的临床诊断可能不明显。本研究的目的是描述抗 Th/To 自身抗体阳性的ILD 患者的肺功能表现和演变。
本方案纳入了抗 Th/To 自身抗体阳性的 ILD 患者。记录了基线临床特征，并进行生存分析，以确定与较差生存相关的危险因素。
共纳入了 52 例抗 Th/To 自身抗体阳性的 ILD 患者。仅有 21%的患者符合 ACR/EULAR 2013 系统性硬化症分类标准，63.4%符合 IPAF ATS/ERS 2015 标准。随访期间有 25%的患者死亡。呼吸衰竭是主要死亡原因。29 例（56%）患者为其他标志性 SSc 自身抗体阳性。最常见的 HRCT 模式是非特异性间质性肺炎（NISP）。生存与收缩期肺动脉压（sPAP）、男性和 HRCT 纤维化程度密切相关；此外，与仅抗 Th/To 阳性的患者相比，其他标志性 SSc 自身抗体阳性的患者生存更差。他们中有 76%表现为纤维性进行性肺病，FVC 绝对下降至少 5%。
只有一小部分抗 Th/To 阳性的 ILD 患者符合 SSc 分类标准；然而，大多数符合 IPAF 标准。很大一部分患者表现为进行性纤维性肺病。生存与 sPAP、肺部疾病的严重程度以及其他标志性 SSc 自身抗体的存在有关。
