Antisynthetase syndrome (ASS) is an overlap connective tissue disease characterized by the presence of myositis-specific autoantibodies directed against tRNA-synthetases. Clinical manifestations are myositis, interstitial lung disease (ILD), Raynaud's phenomenon, mechanic's hands and polyarthritis. Clinical presentation varies between ASS patients. ASS is potentially life threatening due to lung involvement, especially in rapidly progressive forms. Anti-histidyl-tRNA synthetase (anti-Jo1) antibodies are the most frequently detected antibodies in ASS (60 % of patients). Anti-threonyl-tRNA synthetase (anti-PL7) and alanyl-tRNA synthetase (anti-PL12) antibodies are each detected in 10 % of patients approximatively. Anti-tRNA-synthetases antibodies are mutually exclusive. Clinical heterogeneity of ASS patients appears to be associated with specific autoantibodies profile. Patients with anti-Jo1 antibodies have a more systemic presentation (especially with muscle involvement), whereas patients with anti-PL7 or anti-PL12 antibodies have more frequent and isolated ILD. If anti-PL7 and anti-PL12 antibodies are associated with more severe ILD and poorer survival is still matter of debate. Aims of this study were to compare ILD severity at diagnosis and clinical course in patients with ASS according to antisynthetase autoantibodies types.
Study Type
OBSERVATIONAL
Enrollment
200
Follow-up of clinical data, laboratory tests, radiological data and pulmonary function tests
Number of patients of rapidly progressive (RP)- ILD
Decrease of at least 10 percent of Forced Vital Capacity (FVC) OR Decrease of at least 5 % of FVC WITH Clinical worsening and/or ILD extension on CT-scan OR Decrease of at least 15 % of Diffusing Capacity of lung for Carbon Monoxide (DLCO) at 3 months of follow-up
Time frame: 3 months
Number of patients with severe ILD
Hypoxemia (PaO2 \< 60 mmHg) AND/OR Oxygen delivery at time of diagnosis
Time frame: Baseline
Number of patients with ILD relapse
Clinical worsening AND/OR ILD extension on CT-scan AND/OR Progressive ILD on pulmonary function tests WITH modification of corticosteroids dosage or immunosuppressive drugs during follow-up
Time frame: 3 years and 5 years
Number of patients with chronic respiratory failure
Hypoxemia (PaO2 \< 70 mmHg) in a stable state during follow-up
Time frame: 3 years and 5 years
Rate of patients without death or lung transplant
Time frame: 3 years and 5 years
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