Interstitial Pneumonia With Autoimmune Features: Evaluation of Connective Tissue Disease Incidence During Follow-up

Study Overview
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Brief Summary
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Interstitial lung diseases (ILD) represent a frequent complication of connective tissue diseases (CTDs), especially systemic sclerosis, idiopathic inflammatory myopathies and rheumatoid arthritis. ILD can either occur during CTD course or be the first manifestation of CTDs. Therefore screening patients with ILD for CTD is crucial. In some cases, ILD are associated with clinical and/or serological autoimmune features but not classifiable for CTDs. Evolution of these forms to defined CTDs has never been study. Recently, the European Respiratory Society/American Thoracic Society experts proposed a new term, interstitial pneumonia with autoimmune features or IPAF, to describe these patients according to updated classification criteria. Aims of this study were to compare CTD occurence during follow-up between IPAF and non-IPAF patients in a idiopathic interstitial pneumonia cohort and to identify risk factors of CTD progression in IPAF patients at diagnosis.

Official Title
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Interstitial Pneumonia With Autoimmune Features: Evaluation of Connective Tissue Disease Incidence During Follow-up

Conditions
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Idiopathic Interstitial Pneumonia

Intervention / Treatment
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* Other: Follow-up


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients with a new diagnosis of ILD confirmed by two chest-HRCT 3 months apart Patients with a minimal follow-up duration of 3 years after ILD diagnosis Exclusion Criteria: Patients with a defined CTD at ILD diagnosis Patients with an other ILD etiology identified at diagnosis (i.e. sarcoidosis, hypersensitivity pneumonitis)
Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| IPAF patients<br>IPAF definition according to 2015 ERS/ATS criteria | Other: Follow-up<br>* Clinical data, radiological data and laboratory tests follow-up<br>|
| non-IPAF patients<br> | Other: Follow-up<br>* Clinical data, radiological data and laboratory tests follow-up<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| CTD incidence | CTD incidence according to classification criteria: rheumatoid arthritis (2010 ACR/EULAR criteria), systemic erythematosus lupus (2019 ACR/EULAR criteria), Sjögren syndrome (2016 ACR/EULAR criteria), systemic sclerosis (2013 ACR/EULAR criteria), idiopathic inflammatory myopathies (2017 ACR/EULAR criteria) and mixed connective tissue disease (modified Sharp criteria or Alarcon-Segovia criteria or Kasukawa criteria) | After 3 years of follow-up | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| IPAF clinical domain criteria | mechanic hands, Gottron's sign, distal digital tip ulceration, inflammatory arthritis or polyarticular joint stiffness > 60mn, telangiectasia, Raynaud's phenomenon, unexplained digital oedema | Baseline | 
| IPAF serological domain criteria | ANA titre and pattern, RF, anti-CCP, anti-dsDNA, anti-Ro, anti-La, anti-ribonucleoprotein, anti-Smith, anti-Scl70, anti-tRNA synthetase, anti-PM-Scl, anti-MDA5 | Baseline | 
| IPAF morphological domain criteria | NSIP, and/or OP, or LIP radiology pattern by HRCT | Baseline | 
| ILD severity | PFT (pulmonary function test): FVC, FEV1, DLCO (percentages of predicted values) | Baseline, 6 months of follow-up and at the last visit | 
| Survival rate |  | After 3 years and 5 years of follow-up | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Connective tissue disease, Interstitial lung disease, Interstitial pneumonia with autoimmune features