Evaluation of Antigen-specific T Cells in Patients With Antisynthetase Syndrome and Interstitial Lung Disease

Study Overview
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Brief Summary
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Antisynthetase syndrome (AS) is a rare overlapping myositis characterized by cellular and humoral autoimmune responses directed against aminoacyl-tRNA synthetases. Intesrtitial lung disease (ILD) is a leading cause of mortality in antisynthetase syndrome. Recently, antigen-specific IFN-γ+ CD4+ T cells have been identified in bronchoalveolar fluid (BAL) of patients with antisynthetase syndrome and ILD. Elevated levels of IL1β, IL12, IL18, TNFα, IL17A, IL22 have also been detected in peripheral blood of AS patients, especially those with progressive ILD. Implication of innate lymphoid cells (ILC) and mucosal-associated invariant T cells (MAIT) have not yet been studied in patients with AS. Targeted therapies against Th1 and Th17 cells may represent a promising treatment in patients AS patients with ILD. Investigators suppose that antigen-specific Th1 and Th17 cells, ILC and MAIT at ILD diagnosis are associated with ILD severity at diagnosis and could predict treatment response at 6 months. The main objective is to study the correlation between BAL antigen-specific Th1 and Th17 cells at ILD diagnosis and clinical evolution after 6 months of treatment according to initial ILD severity.

Official Title
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Evaluation of Antigen-specific Th1 and T17 Cells, ILC and MAIT in Patients With Antisynthetase Syndrome and Interstitial Lung Disease

Conditions
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Antisynthetase Syndrome

Intervention / Treatment
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* Diagnostic Test: BAL antigen-specific Th1 cells and Th17 cells, ILC and MAIT


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patient with new diagnosis of AS with ILD Exclusion Criteria: Patient with ILD differential diagnosis Corticosteroid treatment, immunosuppresive or immunomodulatory drugs in the past 3 months before diagnosis

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| AS patients with ILD<br>New diagnosis of patients with AS syndrome and ILD | Diagnostic Test: BAL antigen-specific Th1 cells and Th17 cells, ILC and MAIT<br>* BAL antigen-specific Th1 cells and Th17 cells, ILC and MAIT<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| BAL antigen-specific Th1 and Th17 cells | BAL antigen-specific Th1 and Th17 cells percentages among BAL total CD4+ T cells | baseline (J0) | 
| FVC relative change | Relative change of FVC percentage | within 6 months after diagnosis | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| BAL antigen-specific Th1 and Th17 cells | BAL antigen-specific Th1 and Th17 cells percentages among total BAL CD4+ T cells | baseline (J0) | 
| FVC | FVC percentage at ILD diagnosis | baseline (J0) | 
| FVC absolute change | Absolute change of FVC percentage | within 6 months after diagnosis | 
| Global activity | MDAAT score | baseline (J0) |