Incidence, Clinical Characteristics and Evaluation of Prognostic and Diagnostic Markers of Interstitial Pneumonia With Autoimmune Features (IPAF) - a Multicenter Prospective Study
试验速览
- 阶段
- 不适用
- 状态
- 尚未招募
- 入组人数
- 210
- 试验地点
- 7
- 主要终点
- Identification of IPAF diagnostic markers
研究概览
简要总结
Interstitial pneumonia with autoimmune features (IPAF) was defined in 2015 by the Working Group of the European Respiratory Society (ERS) and the American Thoracic Society (ATS) as interstitial pneumonia with some clinical and/or serological features suggesting presence of an underlying autoimmune disorder. However, ofiicial criteria for diagnosis of an autoimmune disease are not met.
Aims of the study:
- Determine the incindence of IPAF in comparison with interstitial lung diseases (ILDs) and classic autoimmune diseases (ADs) in polish pulmonological centers.
- Clinical, serological, functional and radiological and histopathological characteristics of IPAF patients.
- Analysis of diagnostic strategies towards specific IPAF subgroups.
- Characterictics of potencial diagnostic, predictive and prognostic features of IPAF.
- Prospective assessment of IPAF patients in the courseof 5 years in order to determine stability of the diagnosis and potential progression to other diseases, e.g. ADs.
详细描述
The following patient groups will be studied:
-
Study group - IPAF.
-
Control groups:
-
Connective tissue disease associated interstitial lung disease (CTD-ILD) patients: .rheumatoid arthritis - RA, systemic sclerosis - SSc, polymyositis - PM, dermatomyositis - DM, (anti-synthetase syndrome - AS, Sjoegren's syndrome - SjS, mixed connective tissue disease - MCTD ,systemic lupus erythematosus - SLE, diagnosed according to diagnostic criteria issued by European League Against Rheumatism (EULAR) and/or American College of Rheumatology (ACR)
-
Idiopathic interstitial pneumonia group: idiopathic pulmonary fibrosis - IPF, nonspecific interstitial pneumonia - NSIP, cryptogenic organizing pneumonia - COP, acute interstitial pneumonia - AIP; respiratory bronchiolitis associated interstitial lung disease - RB-ILD, desquamative interstitial pneumonia - DIP, lymphocytic interstitial pneumonia - LIP).
Methods Patients will be assessed by a pulmonologist and rheumatologist. Questionnaires regarding clinical symptoms, concomitant diseases and their treatment, disease activity will be fulfilled.
The majority of diagnostic tests and procedures are routinely performed during clinical care.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
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
- •written informed consent to participate in the study
- •diagnosis of one of interstitial lung diseases/autoimmune diseases as described below
排除标准
- •age < 18 years
- •withdrawal od consent
- •pregnancy
- •lactation
- •infectious disease 4 weeks prior
结局指标
主要结局
Identification of IPAF diagnostic markers
时间窗: 10.2019-10.2020
It is still unclear whether there are diagnostic markers specific for IPAF or whether there is a significant difference in concentration of fibrosis biomarkers in IPAF, CTD-ILD and ILD groups. Both blood and BAL biomarkers will be taken under consideration, they include chemokine C-C motif ligand 18 (CXCL18), Surfactant Protein A- (SP-A), Surfactant Protein D (SP-D), Krebs von den Lungen-6 protein (KL-6) and chitotrisidase 1 (CHIT1).
次要结局
未报告次要终点
研究者
Adam Barczyk
Professor, Ph.D., M.D.
Medical University of Silesia