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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.
Full description
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
SPECIFIC OBJECTIVES To characterize patients with ILD associated with each of the most common autoimmune diseases, ANCA-ILD and IPAF.
To describe the prescription patterns according to the underlying autoimmune entity and the severity of the ILD presentation.
To study the lung function changes in patients treated with immunosuppressants and / or antifibrotics.
To report adverse events and long-term safety associated with the use of different prescribed drugs.
To describe the survival of the treatments used. To know the accessibility to prescribed therapies. To know the frequency of use of health resources. To describe the impact of the disease on quality of life and work activity.
STUDY DESIGN The EPIMAR II registry will have a prospective, observational and multicenter design.
The inclusion of new patients for the follow-up cohort (EPIMAR II) will begin in 2021. Patients evaluated and diagnosed in the last 5 years who are currently under follow-up will be included. They will be evaluated at a baseline visit and then annually. Patients in the EPIMAR I cohort who meet the inclusion criteria may be included in this new registry.
POPULATION Patients with a diagnosis of Ai-ILD according to the evaluation of a multidisciplinary team. Patients without respiratory symptoms may be included in the registry and will be classified as subclinical ILD.
INCLUSION CRITERIA
Established or early stage CTD. IPAF according to ATS / ERS 2015 classification criteria. ANCA positivity by immunofluorescence confirmed by ELISA, with or without systemic vasculitis.
EXCLUSION CRITERIA 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)
RECRUITMENT
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Inclusion criteria
Established or early stage CTD 18-30. IPAF according to ATS / ERS 2015 classification criteria 8 ANCA positivity by immunofluorescence confirmed by ELISA, with or without systemic vasculitis 31.
Exclusion criteria
1,000 participants in 1 patient group
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Central trial contact
Juan Enghelmayer, MD; Florencia Vivero, MD
Data sourced from clinicaltrials.gov
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