Evaluation of Interstitial Lung Disease Severity in Patients With Antisynthetase Syndrome According to Specific Autoantibodies Profile

Study Overview
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Brief Summary
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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.

Official Title
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Evaluation of Interstitial Lung Disease Severity in Patients With Antisynthetase Syndrome According to Specific Antisynthetase Antibodies Types

Conditions
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Antisynthetase Syndrome

Intervention / Treatment
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* Other: Follow-up


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients with antisynthetase syndrome according to Connors criteria Exclusion Criteria: None

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Anti-Jo1<br>Patients with anti-Jo1 antibodies | Other: Follow-up<br>* Follow-up of clinical data, laboratory tests, radiological data and pulmonary function tests<br>|
| Anti-PL7<br>Patients with anti-PL7 antibodies | Other: Follow-up<br>* Follow-up of clinical data, laboratory tests, radiological data and pulmonary function tests<br>|
| Anti-PL12<br>Patients with anti-PL12 antibodies | Other: Follow-up<br>* Follow-up of clinical data, laboratory tests, radiological data and pulmonary function tests<br>|
| Anti-EJ<br>Patients with anti-EJ antibodies | Other: Follow-up<br>* Follow-up of clinical data, laboratory tests, radiological data and pulmonary function tests<br>|
| Anti-OJ<br>Patients with anti-OJ antibodies | Other: Follow-up<br>* Follow-up of clinical data, laboratory tests, radiological data and pulmonary function tests<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | 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 | 

Secondary Outcome Measures

| Outcome Measure | 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 | 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 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Interstitial lung disease