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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Anti-Jo1 | Patients with anti-Jo1 antibodies |
| |
| Anti-PL7 | Patients with anti-PL7 antibodies |
| |
| Anti-PL12 | Patients with anti-PL12 antibodies |
| |
| Anti-EJ | Patients with anti-EJ antibodies |
| |
| Anti-OJ | Patients with anti-OJ antibodies |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Follow-up | Other | Follow-up of clinical data, laboratory tests, radiological data and pulmonary function tests |
|
| Measure | Description | Time Frame |
|---|---|---|
| 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 | 3 months |
| Number of patients with severe ILD | Hypoxemia (PaO2 < 60 mmHg) AND/OR Oxygen delivery at time of diagnosis | Baseline |
| Measure | Description | Time Frame |
|---|---|---|
| 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 | 3 years and 5 years |
| Number of patients with chronic respiratory failure |
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Inclusion Criteria:
Exclusion Criteria:
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Patients with antisynthetase syndrome
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Paul DECKER | Contact | +33383157240 | p.decker@chru-nancy.fr |
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| ID | Term |
|---|---|
| C537778 | Antisynthetase syndrome |
| D017563 | Lung Diseases, Interstitial |
| ID | Term |
|---|---|
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
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Hypoxemia (PaO2 < 70 mmHg) in a stable state during follow-up |
| 3 years and 5 years |
| Rate of patients without death or lung transplant | 3 years and 5 years |