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

NCT04924465 | Unknown

• 1 other identifier: 2021PI036
• Study Type: observational
• Target: 200
• Locations: 0 countries
• Sites: N/A

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.

Conditions

Antisynthetase Syndrome

Keywords

Interstitial lung disease

Outcome Measures

Primary Outcomes (2)

• 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 Outcomes (3)

• Number of patients with ILD relapse

  3 years and 5 years

• Number of patients with chronic respiratory failure

  3 years and 5 years

• Rate of patients without death or lung transplant

  3 years and 5 years

Study Arms (5)

Anti-Jo1

Patients with anti-Jo1 antibodies

Other: Follow-up

Anti-PL7

Patients with anti-PL7 antibodies

Other: Follow-up

Anti-PL12

Patients with anti-PL12 antibodies

Other: Follow-up

Anti-EJ

Patients with anti-EJ antibodies

Other: Follow-up

Anti-OJ

Patients with anti-OJ antibodies

Other: Follow-up

Interventions

Follow-up OTHER

Follow-up of clinical data, laboratory tests, radiological data and pulmonary function tests

Anti-EJ; Anti-Jo1; Anti-OJ; Anti-PL12; Anti-PL7

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Patients with antisynthetase syndrome

You may qualify if:

• Patients with antisynthetase syndrome according to Connors criteria

You may not qualify if:

• None

Sponsors & Collaborators

• Central Hospital, Nancy, France: lead

MeSH Terms

Conditions

Antisynthetase syndrome; Lung Diseases, Interstitial

Condition Hierarchy (Ancestors)

Lung Diseases; Respiratory Tract Diseases

Central Study Contacts

Paul DECKER

CONTACT

+33383157240; p.decker@chru-nancy.fr

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: May 8, 2021
• First Posted: June 14, 2021
• Study Start: June 1, 2021
• Primary Completion: June 1, 2023
• Study Completion: December 1, 2023
• Last Updated: June 14, 2021

Record last verified: 2021-05