Antisynthetase Syndrome
Conditions
Brief summary
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.
Interventions
DIAGNOSTIC_TESTBAL antigen-specific Th1 cells and Th17 cells, ILC and MAIT
BAL antigen-specific Th1 cells and Th17 cells, ILC and MAIT
Sponsors
Central Hospital, Nancy, France
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| FVC relative change | within 6 months after diagnosis | Relative change of FVC percentage |
| BAL antigen-specific Th1 and Th17 cells | baseline (J0) | BAL antigen-specific Th1 and Th17 cells percentages among BAL total CD4+ T cells |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| FVC absolute change | within 6 months after diagnosis | Absolute change of FVC percentage |
| Global activity | baseline (J0) | MDAAT score |
| BAL antigen-specific Th1 and Th17 cells | baseline (J0) | BAL antigen-specific Th1 and Th17 cells percentages among total BAL CD4+ T cells |
| FVC | baseline (J0) | FVC percentage at ILD diagnosis |
Other
| Measure | Time frame | Description |
|---|---|---|
| BAL MAIT | baseline (J0) | BAL MAIT percentage among total BAL T cells |
| BAL ILC | baseline (J0) | BAL ILC percentage among total BAL lymphoid cells |
Countries
France
Contacts
Primary ContactPaul Decker, MD
Outcome results
None listed