Evaluation of Circulating Neutrophils in Antisynthetase Syndrome

Recruiting

• Conditions: Antisynthetase Syndrome
• Interventions: Diagnostic Test: circulating neutrophils

• Registration Number: NCT05989399
• Lead Sponsor: Central Hospital, Nancy, France

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 study the correlation between the level of circulating neutrophils 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 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.

Detailed Description

Not available

Study Timeline

• Study First Submitted: 2023-08-04
• Study First Submitted QC: 2023-08-04
• Study First Posted: 2023-08-14
• Study Start: 2024-01-01
• Status Verified: 2024-08
• Last Update Submitted: 2024-08-26
• Last Update Posted: 2024-08-27
• Primary Completion: 2025-10-31
• Study Completion: 2025-10-31

Recruitment & Eligibility

• Status: RECRUITING
• Sex: All
• Target Recruitment: 150

Inclusion Criteria

• Patients with ASyS diagnosis according to Connors criteria

Exclusion Criteria

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

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
ASyS patients	circulating neutrophils	Patients with antisynthetase syndrome

Primary Outcome Measures

Name	Time	Method
number of patients with global severity	baseline (J0)	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
circulating neutrophils level > 7000/mm3	baseline (J0)

Secondary Outcome Measures

Name	Time	Method
CK level	baseline (J0)
FVC	baseline (J0)	FVC percentage
DLCO	baseline (J0)	DLCO percentage
circulating neutrophils level	12 months
BAL fluid neutrophils level	baseline (J0)
grade of MRC muscle testing	baseline (J0)

Trial Locations

Locations (1)

CHU Nancy; 🇫🇷 Nancy, France