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

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

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.

Study Overview

• Status: Not yet recruiting
• Conditions: Antisynthetase Syndrome
• Intervention / Treatment: Other: Follow-up

• Study Type: Observational
• Enrollment (Anticipated): 200

Contacts and Locations

• Study Contact: Name: Paul Decker; Phone Number: +33383157240; Email: p.decker@chru-nancy.fr

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 14 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Patients with antisynthetase syndrome

Description

Inclusion Criteria:

• Patients with antisynthetase syndrome according to Connors criteria

Exclusion Criteria:

• None

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Retrospective

Number of groups / cohorts

5

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Anti-Jo1; Patients with anti-Jo1 antibodies	Other: Follow-up; Follow-up of clinical data, laboratory tests, radiological data and pulmonary function tests
Anti-PL7; Patients with anti-PL7 antibodies	Other: Follow-up; Follow-up of clinical data, laboratory tests, radiological data and pulmonary function tests
Anti-PL12; Patients with anti-PL12 antibodies	Other: Follow-up; Follow-up of clinical data, laboratory tests, radiological data and pulmonary function tests
Anti-EJ; Patients with anti-EJ antibodies	Other: Follow-up; Follow-up of clinical data, laboratory tests, radiological data and pulmonary function tests
Anti-OJ; Patients with anti-OJ antibodies	Other: Follow-up; Follow-up of clinical data, laboratory tests, radiological data and pulmonary function tests

What is the study measuring?

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

Collaborators and Investigators

• Sponsor: Central Hospital, Nancy, France

Study record dates

Study Major Dates

• Study Start (Anticipated): June 1, 2021
• Primary Completion (Anticipated): June 1, 2023
• Study Completion (Anticipated): December 1, 2023

Study Registration Dates

• First Submitted: May 8, 2021
• First Submitted That Met QC Criteria: June 10, 2021
• First Posted (Actual): June 14, 2021

Study Record Updates

• Last Update Posted (Actual): June 14, 2021
• Last Update Submitted That Met QC Criteria: June 10, 2021
• Last Verified: May 1, 2021

More Information

Terms related to this study

• Keywords: Interstitial lung disease
• Additional Relevant MeSH Terms: Pathologic Processes; Nervous System Diseases; Respiratory Tract Diseases; Disease; Musculoskeletal Diseases; Muscular Diseases; Neuromuscular Diseases; Syndrome; Lung Diseases; Myositis; Lung Diseases, Interstitial
• Other Study ID Numbers: 2021PI036

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No