Characteristics of Patients With Autoimmune Interstitial Lund Disease From Argentina
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 发起方
- 入组人数
- 1,000
- 试验地点
- 1
- 主要终点
- Mortality
研究概览
简要总结
Interstitial lung diseases (ILD) constitute a group of entities characterized by inflammation and/or fibrosis of the lung parenchyma.
In recent years, with the advent of new diagnostic tools and therapeutic options, multidisciplinary evaluation is essential, since it optimizes the interpretation of each case and the quality of care for these pathologies Consensus for the identification and management of ILD associated with SSc (ILD-SSc) is the only guideline published at present. In the others autoimmune ILD (Ai-ILD), screening, diagnosis, treatment and follow-up strategies are usually performed according to the criteria of the treating medical team. Guidelines regarding the follow-up and indication of immunosuppressive and antifibrotic treatment are lacking. Many questions on the horizon of the Ai-ILD should be answered as better quality evidence emerges from studies with a greater number of patients and better methodological design.
详细描述
Autoimmune ILD (Ai-ILD) include ILD associated with connective tissue disease (CTD), ILD associated with antineutrophil cytoplasmic antibodies (ANCA-ILD) and interstitial pneumonia with autoimmune findings (IPAF).
PRIMARY OBJECTIVE To describe the baseline sociodemographic, clinical, serological, functional, radiological and treatment characteristics of patients with Ai-ILD and in the 5-year follow-up.
GENERAL OBJECTIVES
- To know the frequency of subclinical Ai-ILD and its evolution.
- To identify risk factors associated with the progression of Ai-ILD.
- To characterize the subgroup of patients with progressive fibrosing phenotype.
- To describe patterns of treatments used with Ai-ILD.
- To describe the most frequent adverse drug events.
- To evaluate global mortality and the factors associated with annual and 5-year survival.
SPECIFIC OBJECTIVES To characterize patients with ILD associated with each of the most common autoimmune diseases, ANCA-ILD and IPAF.
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
- •Age ≥ 18 years old
- •Diagnosis of ILD within the last 5 years according to the criteria of the multidisciplinary team composed of at least one pulmonologist and one rheumatologist, with or without respiratory symptoms.
- •ILD defined by the presence of ground glass opacities and / or peribronchovascular or airspace consolidations and / or reticulations and / or traction bronchiectasis and / or honeycomb on high-resolution computed tomography (HRCT) within the last 12 months 17 .
- •One of the following three criteria (see annex 1):
- •Established or early stage CTD 18-
- •IPAF according to ATS / ERS 2015 classification criteria 8 ANCA positivity by immunofluorescence confirmed by ELISA, with or without systemic vasculitis
- •Spirometry performed within the last 6 months before entering the registry.
- •The participant must sign the informed consent voluntarily.
排除标准
- •Not being able to perform the clinical follow-up or the complementary studies required in the protocol.
- •Not being able to be evaluated by a multidisciplinary team; at least a rheumatologist plus a pulmonologist.
- •ILD associated with another non-autoimmune etiology according to the criteria of the multidisciplinary team (eg, occupational diseases, toxic)
结局指标
主要结局
Mortality
时间窗: 5 years
Number of patients that Survival at end of the study
Fibrosis in Computed tomography
时间窗: baseline and annual visits
Change in Computed tomography in relation to fibrotic involvement, measured by the same operator
Pulmonary test functionals
时间窗: baseline and annual visits
Change in functional tests over time (measurement of FVC and DLCO)
次要结局
- Safety of the treatments used(5 years)