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
Study Type
OBSERVATIONAL
Enrollment
300
evaluation of SSc phenotypes
CHU Angers
Angers, France
CHU Brest
Brest, France
CH Dunkerque
Dunkirk, France
CHU Grenoble
Grenoble, France
CHU Lille
Lille, France
Hospices Civils de Lyon
Lyon, France
AP-HM
Marseille, France
CHU Nice
Nice, France
APHP
Paris, France
CHU Poitiers
Poitiers, France
...and 4 more locations
diagnosis time
duration between date of first symptom (excluding Raynaud's phenomenon) and SSc diagnosis
Time frame: baseline (J0)
number of patients with scleroderma
sine scleroderma (no scleroderma), limited scleroderma or diffuse scleroderma
Time frame: baseline (J0)
number of patients with raynaud's phenomenon
Time frame: baseline (J0)
number of patients with digital ulcers
Time frame: baseline (J0), 3 years of follow-up and through study completion (an average of 5 years)
number of patients with calcinosis
Time frame: baseline (J0), 3 years of follow-up and through study completion (an average of 5 years)
number of patients with telangiectases
Time frame: baseline (J0)
number of patients with articular involvement
Time frame: baseline (J0), 3 years of follow-up and through study completion (an average of 5 years)
number of patients with muscular involvement
Time frame: baseline (J0), 3 years of follow-up and through study completion (an average of 5 years)
number of patients with cardiac involvement
Time frame: baseline (J0), 3 years of follow-up and through study completion (an average of 5 years)
number of patients with interstitial lung disease
Time frame: baseline (J0), 3 years of follow-up and through study completion (an average of 5 years)
number of patients with pulmonary arterial hypertension
Time frame: baseline (J0), 3 years of follow-up and through study completion (an average of 5 years)
number of patients with scleroderma renal crisis
Time frame: baseline (J0), 3 years of follow-up and through study completion (an average of 5 years)
number of patients with gastrointestinal involvement
Time frame: baseline (J0), 3 years of follow-up and through study completion (an average of 5 years)
modified Rodnan skin score
Time frame: baseline (J0), 3 years of follow-up and through study completion (an average of 5 years)
forced vital capacity (FVC)
%predicted FVC values
Time frame: baseline (J0), 3 years of follow-up and through study completion (an average of 5 years)
diffusing capacity for carbon monoxide (DLCO)
%predicted DLCO values
Time frame: baseline (J0), 3 years of follow-up and through study completion (an average of 5 years)
rate of patients without death
Time frame: 3 years and 5 years of follow-up
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