Evaluation of Interstitial Lung Disease Severity in Patients With Antisynthetase Syndrome According to Specific Antisynthetase Antibodies Types
试验速览
- 阶段
- 不适用
- 发起方
- 入组人数
- 200
- 主要终点
- Number of patients of rapidly progressive (RP)- ILD
研究概览
简要总结
Antisynthetase syndrome (ASS) is an overlap connective tissue disease characterized by the presence of myositis-specific autoantibodies directed against tRNA-synthetases. Clinical manifestations are myositis, interstitial lung disease (ILD), Raynaud's phenomenon, mechanic's hands and polyarthritis. Clinical presentation varies between ASS patients. ASS is potentially life threatening due to lung involvement, especially in rapidly progressive forms. Anti-histidyl-tRNA synthetase (anti-Jo1) antibodies are the most frequently detected antibodies in ASS (60 % of patients). Anti-threonyl-tRNA synthetase (anti-PL7) and alanyl-tRNA synthetase (anti-PL12) antibodies are each detected in 10 % of patients approximatively. Anti-tRNA-synthetases antibodies are mutually exclusive.
Clinical heterogeneity of ASS patients appears to be associated with specific autoantibodies profile. Patients with anti-Jo1 antibodies have a more systemic presentation (especially with muscle involvement), whereas patients with anti-PL7 or anti-PL12 antibodies have more frequent and isolated ILD. If anti-PL7 and anti-PL12 antibodies are associated with more severe ILD and poorer survival is still matter of debate.
Aims of this study were to compare ILD severity at diagnosis and clinical course in patients with ASS according to antisynthetase autoantibodies types.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Retrospective
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Patients with antisynthetase syndrome according to Connors criteria
排除标准
- 未提供
结局指标
主要结局
Number of patients of rapidly progressive (RP)- ILD
时间窗: 3 months
Decrease of at least 10 percent of Forced Vital Capacity (FVC) OR Decrease of at least 5 % of FVC WITH Clinical worsening and/or ILD extension on CT-scan OR Decrease of at least 15 % of Diffusing Capacity of lung for Carbon Monoxide (DLCO) at 3 months of follow-up
Number of patients with severe ILD
时间窗: Baseline
Hypoxemia (PaO2 \< 60 mmHg) AND/OR Oxygen delivery at time of diagnosis
次要结局
- Rate of patients without death or lung transplant(3 years and 5 years)
- Number of patients with ILD relapse(3 years and 5 years)
- Number of patients with chronic respiratory failure(3 years and 5 years)