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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| AS patients with ILD | New diagnosis of patients with AS syndrome and ILD |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| BAL antigen-specific Th1 cells and Th17 cells, ILC and MAIT | Diagnostic Test | BAL antigen-specific Th1 cells and Th17 cells, ILC and MAIT |
|
| 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 |
| 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 |
| Measure | Description | Time Frame |
|---|---|---|
| BAL ILC | BAL ILC percentage among total BAL lymphoid cells | baseline (J0) |
| BAL MAIT | BAL MAIT percentage among total BAL T cells | baseline (J0) |
Inclusion Criteria:
Exclusion Criteria:
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Patients with AS and ILD
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Paul Decker, MD | Contact | +33383157240 | p.decker@chru-nancy.fr |
| Name | Affiliation | Role |
|---|---|---|
| Paul Decker, MD | Central Hospital, Nancy, France | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Bernard Bonnotte | Recruiting | Dijon | France |
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| baseline (J0) |
| FVC absolute change | Absolute change of FVC percentage | within 6 months after diagnosis |
| Global activity | MDAAT score | baseline (J0) |
| Julien Campagne | Recruiting | Metz | France |
|
| Paul Decker | Recruiting | Nancy | France |
|
| Olivier Benveniste | Not yet recruiting | Paris | France |
|
| Loïs Bolko | Recruiting | Reims | France |
|
| Alain Meyer | Recruiting | Strasbourg | France |
|
| ID | Term |
|---|---|
| C537778 | Antisynthetase syndrome |
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