抗合成酶综合征合并间质性肺疾病患者的临床特征和预后因素的分析

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

摘要: 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 < 0.01)。③ ASS-ILD最常见的ILD类型是NSIP-OP型(42例，37.83%)。抗PL-7和抗EJ抗体亚组中NSIP的发生率显著高于抗Jo-1和抗PL-12抗体亚组(P < 0.01)。抗PL-12抗体亚组的RP-ILD发生率最高，但各亚组间没有显著差异(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。

Abstract: 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 < 0.01). ③ The most common ILD type of ASS-ILD is NSIP-OP type (42 cases, 37.83%). The incidence of NSIP in the anti-PL-7 and anti-EJ antibody subgroups was significantly higher than that in the anti-Jo-1 and anti-PL-12 antibody subgroups (P < 0.01). The incidence of RP-ILD was highest in the anti-PL-12 antibody subgroup, but there was no significant difference between the subgroups (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.