Analysis of Clinical Characteristics and Prognostic Factors in Patients with Anti Synthetase Syndrome Complicated with Interstitial Lung Disease

目的：探讨抗合成酶综合征(ASS)合并间质性肺病(ILD)患者的临床特征和影像学进展的预后因素。方法：收集111例确诊为ASS-ILD的住院患者的人口学、临床资料、血清学指标、影像学资料及肺功能的表现。随访1年内(至少半年)，根据高分辨率CT (HRCT)影像学的变化分为三组：好转组，稳定组和恶化组。采用χ2检验、Fisher确切概率法比较不同抗氨酰tRNA合成酶(ARS)抗体阳性的ASS-ILD患者临床特征的差异，应用单变量Logistic回归分析评估影像学变化的相关性因素。结果：① 111例ASS-ILD患者中，70名抗Jo-1抗体阳性，17名抗PL-7抗体阳性，15名抗EJ抗体阳性，9名抗PL-12抗体阳性。高雪氏疹是ASS-ILD患者皮肤病变中最常见表现。ASS-ILD最常见的首发症状是呼吸困难。关节炎是抗Jo-1抗体阳性组中最常见的首发症状。抗PL-12抗体阳性组呼吸困难显著高于其他亚组(P = 0.024)。抗PL-12和抗EJ抗体组以咳嗽作为首发症状的发生率显著高于其他亚组(P = 0.000)。② 不同亚组ASS-ILD患者的血清学特征比较，治疗前基线数据显示，抗PL-7和抗PL-12抗体亚组的CD16 + CD56 + (NK)细胞数量显著减少，具有统计学差异(P P P = 0.075)。④ 影像学进展方面，恶化组ASS-ILD患者B淋巴细胞初始数量显著增加，好转组患者糖皮质激素初始剂量更高。统计数据显示CD3-CD19+细胞初始数量(OR = 1.0013, P = 0.014)和初始糖皮质激素用量(OR = 0.9603, P = 0.04)与ILD影像学转归相关。结论：不同抗合成酶抗体亚型的ASS-ILD患者的临床特征不同。B淋巴细胞水平的增加及早期高剂量糖皮质激素强化治疗可能是ASS-ILD患者影像学恶化的独立危险因素。早期高剂量糖皮质激素的强化治疗可能有效改善ILD的影像学预后。Objective: Exploring the clinical characteristics and prognostic factors of imaging progression in patients with antisynthetase syndrome (ASS) complicated with interstitial lung disease (ILD). Methods: Collect demographic, clinical, serological, imaging, and pulmonary function data of 111 hospitalized patients diagnosed with ASS-ILD. Within one year (at least six months) of follow-up, patients were divided into three groups based on changes in high-resolution CT (HRCT) imaging: improvement group, stable group, and deterioration group. Using chi square test and Fisher’s exact probability method to compare the differences in clinical characteristics of ASS-ILD patients with different levels of anti aminoacyl tRNA synthetase (ARS) antibody positivity, and applying univariate cumulative logistic regression analysis to evaluate the correlation factors of imaging changes. Results: ① Among 111 ASS-ILD patients, 70 were positive for anti-Jo-1 antibodies, 17 were positive for anti-PL-7 antibodies, 15 were positive for anti-EJ antibodies, and 9 were positive for anti-PL-12 antibodies. Gottron papules is the most common manifestation of skin lesions in ASS-ILD patients. The most common initial symptom in ASS-ILD patients is dyspnea. Arthritis is the most common initial symptom in the anti-Jo-1 antibody positive group. The respiratory distress in the anti-PL-12 antibody positive group was significantly higher than that in other subgroups (P = 0.024). The incidence of cough as the initial symptom was significantly higher in the anti-PL-12 and anti-EJ antibody groups than in other subgroups (P = 0.000). ② Comparison of serological characteristics among different subgroups of ASS-ILD patients. Baseline data before treatment showed a significant decrease in the number of CD16 + CD56 + (NK) cells in the anti-PL-7 and anti-PL-12 antibody subgroups, with statistical differences (P P P = 0.075). ④ In terms of imaging progress, the initial number of CD3-CD19+cells significantly increased in the worsening group of ASS-ILD patients, while the initial dose of glucocorticoids was higher in the improving group of patients. Statistical data shows that the initial number of B lymphocyte cells (OR = 1.0013, P = 0.014) and the initial dosage of glucocorticoids (OR = 0.9603, P = 0.04) are correlated with the imaging outcome of ILD. Conclusions: The clinical characteristics of ASS-ILD patients with different subtypes of antisynthetase antibodies are different. The increase in B lymphocyte cell levels and early high-dose glucocorticoid enhanced therapy may be independent risk factors for imaging deterioration in ASS-ILD patients. Early high-dose glucocorticoid enhanced therapy may effectively improve the imaging prognosis of ILD.