Antisynthetase syndrome (AS) is a rare overlapping myositis characterized by cellular and humoral autoimmune responses directed against aminoacyl-tRNA synthetases. A pathogenic role of neutrophils was described during inflammatory myopathies, with increased netosis correlated with disease activity and muscle damage. Higher number of alveolar neutrophils was observed in patients with rapidly progressive forms of interstitial lung disease. Peripheral neutrophils could represent a simple biomarker of severity and activity in patients with antisynthetase syndrome. The main objective is to compare circulating neutrophils between severe and non severe patients with antisynthetase syndrome. Secondary objectives are: 1) to evaluate correlation between circulating neutrophils and organ-specific severity, 2) to compare circulating neutrophils at time of diagnosis and circulating neutrophils after 6 months of treatment in patients with antisynthetase syndrome.
Study Type
OBSERVATIONAL
Enrollment
40
CHU Nancy
Vandœuvre-lès-Nancy, France
Number of circulating neutrophils
circulating neutrophils values (number of cells/mm3)
Time frame: at diagnosis (Day 0)
Global severity
composite criteria of global severity: severe interstitial lung disease (ILD) and/or severe muscular disease
Time frame: at diagnosis (Day 0)
Circulating neutrophils change over time
circulating neutrophils values (number of cells/mm3)
Time frame: at 6 months of follow-up
ILD severity
%predicted FVC values in patients with ILD
Time frame: at diagnosis (Day 0)
ILD severity
%predicted DLCO values in patients with ILD
Time frame: at diagnosis (Day 0)
Muscular severity
manual muscle testing (MMT) grading in patients muscle involvement
Time frame: at diagnosis (Day 0)
Muscular severity
CPK values (U/L) in patients muscle involvement
Time frame: at diagnosis (Day 0)
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