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Antisynthetase syndrome (ASyS) is a rare and heteregeneous overlapping connective tissue disease, characterized by myositis, interstitial lung disease (ILD), joint involvement, Raynaud's phenomenon and cutaneous manifestations ("mechanic's hands"). Over 50% of patients develop ILD, which is the leading cause of death.
The role of neutrophils - innate immune cells involved in inflammatory processes and induced in particular by cytokines of the Th17 pathway - during AS is unknown. Direct pathogenic role of neutrophils has been described during idiopathic inflammatory myopathies, with an increase of netosis correlated with disease activity and muscle damage. During ASyS, a higher number of alveolar neutrophils has been observed in patients with rapidly progressive ILD.
There are few data on the specific evaluation of circulating neutrophils in ASyS. Investigators suppose that circulating neutrophils level could represent a simple and accessible severity biomarker in patients with ASyS.
The main objective is to evaluate the diagnostic performance of the circulating neutrophils level (> 7000/mm3) at diagnosis on ASyS severity.
The secondary objectives are:
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| ASyS patients | Patients with antisynthetase syndrome |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| circulating neutrophils | Diagnostic Test | evaluation of circulating neutrophils level |
|
| Measure | Description | Time Frame |
|---|---|---|
| circulating neutrophils level > 7000/mm3 | baseline (J0) | |
| number of patients with global severity | global severity defined as presence of severe ILD (PaO2 < 600 mmHg or rapidly progressive ILD), and/or presence of severe myositis (bedrest, or MMT8 score < 30, or MRC muscle testing < 3/5, or swallowing disorers, or dysphagia) and/or presence of myocarditis on cardiac MRI | baseline (J0) |
| Measure | Description | Time Frame |
|---|---|---|
| circulating neutrophils level | baseline (J0) | |
| circulating neutrophils/lymphocytes ratio | baseline (J0) | |
| FVC |
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Inclusion Criteria:
Exclusion Criteria:
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Patients with ASyS diagnosis between January 2010 and December 2022
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| Name | Affiliation | Role |
|---|---|---|
| Paul Decker, MD | Central Hospital, Nancy, France | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| CHU Nancy | Nancy | France |
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| ID | Term |
|---|---|
| C537778 | Antisynthetase syndrome |
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FVC percentage |
| baseline (J0) |
| DLCO | DLCO percentage | baseline (J0) |
| grade of MRC muscle testing | baseline (J0) |
| CK level | baseline (J0) |
| circulating neutrophils level | 12 months |
| circulating neutrophils/lymphocytes ratio | 12 months |
| BAL fluid neutrophils level | baseline (J0) |
| BAL fluid neutrophils/lymphocytes ratio | baseline (J0) |