Antisynthetase Syndrome
Conditions
Keywords
Interstitial lung disease
Brief summary
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.
Interventions
OTHERFollow-up
Follow-up of clinical data, laboratory tests, radiological data and pulmonary function tests
Sponsors
Central Hospital, Nancy, France
Study design
Observational model
COHORT
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Patients with antisynthetase syndrome according to Connors criteria
Exclusion criteria
* None
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Number of patients of rapidly progressive (RP)- ILD | 3 months | 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 |
| Number of patients with severe ILD | Baseline | Hypoxemia (PaO2 \< 60 mmHg) AND/OR Oxygen delivery at time of diagnosis |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Number of patients with ILD relapse | 3 years and 5 years | 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 |
| Number of patients with chronic respiratory failure | 3 years and 5 years | Hypoxemia (PaO2 \< 70 mmHg) in a stable state during follow-up |
| Rate of patients without death or lung transplant | 3 years and 5 years | — |
Contacts
Primary ContactPaul DECKER
Outcome results
None listed