Evaluation of Patients With Systemic Sclerosis Without Specific or Associated Autoantibodies
- Conditions
- Systemic Sclerosis
- Interventions
- Other: disease phenotype
- Registration Number
- NCT06412614
- Lead Sponsor
- Central Hospital, Nancy, France
- Brief Summary
Systemic sclerosis (SSc) is a complex systemic autoimmune disease with variable phenotype and prognosis. Autoantibodies are important diagnostic biomarkers in SSc. More than 90% of patients with SSc had anti-nuclear antibodies. Autoantibodies specific to SSc (anti-topoisomerase I antibodies, anti-centromeres, anti-RNA polymerase III, anti-Th/To, anti-fibrillarin, anti-NOR90) or associated with overlap syndromes (anti-RNA polymerase III antibodies -PM/Scl, anti-KU, anti-U1RNP, anti-TRIM21) are detected in most patients. Excluding anti-TRIM21 antibodies, autoantibodies are usually mutually exclusive and are associated with distinct phenotypes.
Around 5 to 10% of patients with SSc have no autoantibodies detectable with routine biological tests. Recently, new autoantibody specificities have been described in SSc (anti-eIF2B, anti-RuvBL1/2, anti-BICD2, anti-U11/U12 RNP antibodies).
"Seronegative" patients could represent new specificities of autoantibodies (unknown or not currently routinely evaluated) associated with different phenotypes of the disease.
Primary objective is to compare the phenotype of patients with systemic sclerosis with or without detectable specific or associated autoantibodies.
Secondary objectives are:
* to determine homogeneous groups of patients with systemic sclerosis without detectable specific or associated autoantibodies
* to compare the phenotype of patients with systemic sclerosis without detectable specific or associated autoantibodies according to anti-nuclear antibodies status
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- NOT_YET_RECRUITING
- Sex
- All
- Target Recruitment
- 300
- Patient with systemic sclerosis defined according to ACR/EULAR 2013 classification criteria
- Patient with a minimum follow-up of 3 years since the diagnosis of systemic sclerosis
- Patient evaluated for the following systemic sclerosis specific and/or associated autoantibodies: anti-topoisomerase I, anti-centromere, anti-RNA polymerase III (RP155 and RP11), anti-Th/To antibodies , anti-fibrillarin, anti-NOR90, anti-PM/Scl, anti-KU, anti-U1RNP and anti-SSA antibodies (independently of antinuclear antibodies status)
- Patient with equivocal results for one or more systemic sclerosis specific and/or associated autoantibodies
- Patient initially negative but with a positive result for systemic sclerosis specific and/or associated autoantibodies during follow-up
Study & Design
- Study Type
- OBSERVATIONAL
- Study Design
- Not specified
- Arm && Interventions
Group Intervention Description SSc patients with specific or associated autoantibodies disease phenotype - SSc patients without specific or associated autoantibodies ("seronegative" patients) disease phenotype -
- Primary Outcome Measures
Name Time Method diagnosis time baseline (J0) duration between date of first symptom (excluding Raynaud's phenomenon) and SSc diagnosis
- Secondary Outcome Measures
Name Time Method number of patients with calcinosis baseline (J0), 3 years of follow-up and through study completion (an average of 5 years) number of patients with scleroderma renal crisis baseline (J0), 3 years of follow-up and through study completion (an average of 5 years) forced vital capacity (FVC) baseline (J0), 3 years of follow-up and through study completion (an average of 5 years) %predicted FVC values
number of patients with scleroderma baseline (J0) sine scleroderma (no scleroderma), limited scleroderma or diffuse scleroderma
diffusing capacity for carbon monoxide (DLCO) baseline (J0), 3 years of follow-up and through study completion (an average of 5 years) %predicted DLCO values
number of patients with muscular involvement baseline (J0), 3 years of follow-up and through study completion (an average of 5 years) number of patients with pulmonary arterial hypertension baseline (J0), 3 years of follow-up and through study completion (an average of 5 years) number of patients with raynaud's phenomenon baseline (J0) number of patients with digital ulcers baseline (J0), 3 years of follow-up and through study completion (an average of 5 years) number of patients with telangiectases baseline (J0) number of patients with articular involvement baseline (J0), 3 years of follow-up and through study completion (an average of 5 years) number of patients with cardiac involvement baseline (J0), 3 years of follow-up and through study completion (an average of 5 years) number of patients with interstitial lung disease baseline (J0), 3 years of follow-up and through study completion (an average of 5 years) modified Rodnan skin score baseline (J0), 3 years of follow-up and through study completion (an average of 5 years) number of patients with gastrointestinal involvement baseline (J0), 3 years of follow-up and through study completion (an average of 5 years) rate of patients without death 3 years and 5 years of follow-up
Trial Locations
- Locations (13)
CHU Angers
🇫🇷Angers, France
CHU Brest
🇫🇷Brest, France
CH Dunkerque
🇫🇷Dunkerque, France
CHU Grenoble
🇫🇷Grenoble, France
Hospices Civils de Lyon
🇫🇷Lyon, France
CHU Lille
🇫🇷Lille, France
APHP
🇫🇷Paris, France
AP-HM
🇫🇷Marseille, France
CHU Reims
🇫🇷Reims, France
CHU Nice
🇫🇷Nice, France
CHU Poitiers
🇫🇷Poitiers, France
CHU Rennes
🇫🇷Rennes, France
Hôpitaux Universitaires de Strasbourg
🇫🇷Strasbourg, France