Evaluation of Circulating Neutrophils in Antisynthetase Syndrome: a Multicenter Retrospective Study
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 发起方
- 入组人数
- 89
- 试验地点
- 2
- 主要终点
- number of patients with global severity
研究概览
简要总结
Antisynthetase syndrome (ASyS) is a rare and heteregeneous overlapping connective tissue disease, characterized by myositis, interstitial lung disease (ILD), joint involvement, Raynaud's phenomenon and cutaneous manifestations ("mechanic's hands"). Over 50% of patients develop ILD, which is the leading cause of death.
The role of neutrophils - innate immune cells involved in inflammatory processes and induced in particular by cytokines of the Th17 pathway - during AS is unknown. Direct pathogenic role of neutrophils has been described during idiopathic inflammatory myopathies, with an increase of netosis correlated with disease activity and muscle damage. During ASyS, a higher number of alveolar neutrophils has been observed in patients with rapidly progressive ILD.
There are few data on the specific evaluation of circulating neutrophils in ASyS. Investigators suppose that circulating neutrophils level could represent a simple and accessible severity biomarker in patients with ASyS.
The main objective is to evaluate the diagnostic performance of the circulating neutrophils level (> 7000/mm3) at diagnosis on ASyS severity.
The secondary objectives are:
- to define a threshold for circulating neutrophils levels at diagnosis allowing to predict ASyS severity and to assess the diagnostic performance of this threshold.
- to define a threshold for the circulating neutrophils/lymphocytes ratio at diagnosis allowing to predict ASyS severity and to assess the diagnostic performance of this threshold.
- to study the correlation between the level of circulating neutrophils and ASyS severity at diagnosis of the disease.
- to study the correlation between the circulating neutrophils/lymphocytes ratio and ASyS severity at diagnosis of the disease.
- to compare the circulating neutrophils level at ASyS diagnosis and after 1 year of treatment.
- to compare the circulating neutrophils/lymphocytes ratio at ASyS diagnosis and after 1 year of treatment.
- to compare patients characteristics according to ASyS severity at diagnosis.
- to compare BAL fluid neutrophils level according to ILD severity at ASyS diagnosis in patients with ILD.
- to compare BAL fluid neutrophils/lymphocytes ratio according to ILD severity at ASyS diagnosis in patients with ILD.
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 ASyS diagnosis according to Connors criteria
排除标准
- •Active infection at ASyS diagnosis
- •Evolutive cancer at ASyS diagnosis
- •Corticosteroid therapy initiation before circulating neutrophils evaluation
研究组 & 干预措施
ASyS patients
Patients with antisynthetase syndrome
干预措施: circulating neutrophils (Diagnostic Test)
结局指标
主要结局
number of patients with global severity
时间窗: baseline (J0)
global severity defined as presence of severe ILD (PaO2 \< 600 mmHg or rapidly progressive ILD), and/or presence of severe myositis (bedrest, or MMT8 score \< 30, or MRC muscle testing \< 3/5, or swallowing disorers, or dysphagia) and/or presence of myocarditis on cardiac MRI
circulating neutrophils level > 7000/mm3
时间窗: baseline (J0)
次要结局
- CK level(baseline (J0))
- FVC(baseline (J0))
- DLCO(baseline (J0))
- circulating neutrophils level(12 months)
- BAL fluid neutrophils level(baseline (J0))
- grade of MRC muscle testing(baseline (J0))
- circulating neutrophils level(baseline (J0))
- circulating neutrophils/lymphocytes ratio(baseline (J0))
- circulating neutrophils/lymphocytes ratio(12 months)
- BAL fluid neutrophils/lymphocytes ratio(baseline (J0))
研究者
Paul DECKER, MD
Principal Investigator
Central Hospital, Nancy, France