Evaluation of Circulating Neutrophils in Antisynthetase Syndrome

NCT05989399 | Completed

• 1 other identifier: 2023PI083
• Study Type: observational
• Target: 89
• Locations: 1 country
• Sites: 1

Brief Summary

Antisynthetase syndrome (ASyS) is a rare and heteregeneous overlapping connective tissue disease, characterized by myositis, interstitial lung disease (ILD), joint involvement, Raynaud's phenomenon and cutaneous manifestations ("mechanic's hands"). Over 50% of patients develop ILD, which is the leading cause of death. The role of neutrophils - innate immune cells involved in inflammatory processes and induced in particular by cytokines of the Th17 pathway - during AS is unknown. Direct pathogenic role of neutrophils has been described during idiopathic inflammatory myopathies, with an increase of netosis correlated with disease activity and muscle damage. During ASyS, a higher number of alveolar neutrophils has been observed in patients with rapidly progressive ILD. There are few data on the specific evaluation of circulating neutrophils in ASyS. Investigators suppose that circulating neutrophils level could represent a simple and accessible severity biomarker in patients with ASyS. The main objective is to evaluate the diagnostic performance of the circulating neutrophils level (\> 7000/mm3) at diagnosis on ASyS severity. The secondary objectives are:

• to define a threshold for circulating neutrophils levels at diagnosis allowing to predict ASyS severity and to assess the diagnostic performance of this threshold.
• to define a threshold for the circulating neutrophils/lymphocytes ratio at diagnosis allowing to predict ASyS severity and to assess the diagnostic performance of this threshold.
• to study the correlation between the level of circulating neutrophils and ASyS severity at diagnosis of the disease.
• to study the correlation between the circulating neutrophils/lymphocytes ratio and ASyS severity at diagnosis of the disease.
• to compare the circulating neutrophils level at ASyS diagnosis and after 1 year of treatment.
• to compare the circulating neutrophils/lymphocytes ratio at ASyS diagnosis and after 1 year of treatment.
• to compare patients characteristics according to ASyS severity at diagnosis.
• to compare BAL fluid neutrophils level according to ILD severity at ASyS diagnosis in patients with ILD.
• to compare BAL fluid neutrophils/lymphocytes ratio according to ILD severity at ASyS diagnosis in patients with ILD.

Conditions

Antisynthetase Syndrome

Outcome Measures

Primary Outcomes (2)

• circulating neutrophils level > 7000/mm3

  baseline (J0)

• number of patients with global severity

  global severity defined as presence of severe ILD (PaO2 \< 600 mmHg or rapidly progressive ILD), and/or presence of severe myositis (bedrest, or MMT8 score \< 30, or MRC muscle testing \< 3/5, or swallowing disorers, or dysphagia) and/or presence of myocarditis on cardiac MRI

  baseline (J0)

Secondary Outcomes (10)

• circulating neutrophils level

  baseline (J0)

• circulating neutrophils/lymphocytes ratio

  baseline (J0)

• FVC

  baseline (J0)

• DLCO

  baseline (J0)

• grade of MRC muscle testing

  baseline (J0)

Study Arms (1)

ASyS patients

Patients with antisynthetase syndrome

Diagnostic Test: circulating neutrophils

Interventions

circulating neutrophils DIAGNOSTIC_TEST

evaluation of circulating neutrophils level

ASyS patients

Eligibility Criteria

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

Study Population

Patients with ASyS diagnosis between January 2010 and December 2022

You may qualify if:

• Patients with ASyS diagnosis according to Connors criteria

You may not qualify if:

• Active infection at ASyS diagnosis
• Evolutive cancer at ASyS diagnosis
• Corticosteroid therapy initiation before circulating neutrophils evaluation

Sponsors & Collaborators

• Central Hospital, Nancy, France: lead

Study Sites (1)

CHU Nancy

Nancy, France

Location

MeSH Terms

Conditions

Antisynthetase syndrome

Study Officials

• Paul Decker, MD

  CHU NANCY

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Principal Investigator

Study Record Dates

• First Submitted: August 4, 2023
• First Posted: August 14, 2023
• Study Start: January 1, 2024
• Primary Completion: January 10, 2026
• Study Completion: January 10, 2026
• Last Updated: April 20, 2026

Record last verified: 2026-04

Data Sharing

• IPD Sharing: Will not share

Locations

FR (1)