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

Central Hospital, Nancy, France

Status: Unknown

Conditions: Antisynthetase Syndrome

Treatments: Other: Follow-up

Study type: Observational

Funder types: Other

Identifiers

NCT04924465

2021PI036

Details and patient eligibility

About

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.

Enrollment: 200 estimated patients

Sex: All

Ages: 18+ years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• Patients with antisynthetase syndrome according to Connors criteria

Exclusion criteria

• None

Trial design

200 participants in 5 patient groups

Anti-Jo1

Description:

Patients with anti-Jo1 antibodies

Treatment:

Other: Follow-up

Anti-PL7

Description:

Patients with anti-PL7 antibodies

Treatment:

Other: Follow-up

Anti-PL12

Description:

Patients with anti-PL12 antibodies

Treatment:

Other: Follow-up

Anti-EJ

Description:

Patients with anti-EJ antibodies

Treatment:

Other: Follow-up

Anti-OJ

Description:

Patients with anti-OJ antibodies

Treatment:

Other: Follow-up