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.
Study Type
OBSERVATIONAL
Enrollment
24
BAL antigen-specific Th1 cells and Th17 cells, ILC and MAIT
Bernard Bonnotte
Dijon, France
RECRUITINGJulien Campagne
Metz, France
RECRUITINGPaul Decker
Nancy, France
RECRUITINGOlivier Benveniste
Paris, France
NOT_YET_RECRUITINGLoïs Bolko
Reims, France
RECRUITINGAlain Meyer
Strasbourg, France
RECRUITINGBAL antigen-specific Th1 and Th17 cells
BAL antigen-specific Th1 and Th17 cells percentages among BAL total CD4+ T cells
Time frame: baseline (J0)
FVC relative change
Relative change of FVC percentage
Time frame: within 6 months after diagnosis
BAL antigen-specific Th1 and Th17 cells
BAL antigen-specific Th1 and Th17 cells percentages among total BAL CD4+ T cells
Time frame: baseline (J0)
FVC
FVC percentage at ILD diagnosis
Time frame: baseline (J0)
FVC absolute change
Absolute change of FVC percentage
Time frame: within 6 months after diagnosis
Global activity
MDAAT score
Time frame: baseline (J0)
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