Registry of Autoimmune Interstitial Lung Disease

Summary

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.

Eligibility

Inclusion Criteria:

* 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 18-30. IPAF according to ATS / ERS 2015 classification criteria 8 ANCA positivity by immunofluorescence confirmed by ELISA, with or without systemic vasculitis 31.

* Spirometry performed within the last 6 months before entering the registry.
* The participant must sign the informed consent voluntarily.

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)

Conditions2

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