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Disease Subsets, Antinuclear Antibody Profile, and Clinical Features in 127 French and 247 US Adult Patients with Systemic Sclerosis
OLIVIER C. MEYER, NOREEN FERTIG, MARY LUCAS, NATHALIE SOMOGYI, and THOMAS A. MEDSGER Jr ABSTRACT. Objective. To investigate the specificities of antinuclear antibodies (ANA) associated with systemic sclerosis (SSc) disease classification and internal organ involvement among patients with SSc of different origins (European and American). Methods. Serum samples from 374 adult patients diagnosed with SSc were studied: 127 French patients (Paris) were compared with 247 US patients (Pittsburgh). Patients were classified into diffuse cutaneous (dc) and limited cutaneous (lc) SSc subsets. Antibodies associated with SSc were determined by protein and/or RNA immunoprecipitation, indirect immunofluorescence, and immunodiffusion. Results. SSc classification differed significantly in the 2 cohorts: lcSSc and overlap patients with lcSSc combined made up 76% of the French series versus 52% of the US group (p < 0.0001). The frequency of anti-RNA polymerase III antibody was significantly increased in US patients compared with French patients (p < 0.0001). The frequency of anti-topoisomerase I (topo I) antibody was significantly increased among French patients (p < 0.0048). Anti-topo I-positive French SSc patients were less likely to have dcSSc (38% vs 65%) and more likely to have milder disease than US anti-topo I-positive patients. The French dcSSc patients had lower proportions of joint/tendon manifestations and renal crisis (7% vs 17%), but more often had radiographic evidence of pulmonary fibrosis (57% vs 30%). French lcSSc patients had a lower frequency of pulmonary arterial hypertension than US lcSSc patients (9% vs 31%; p = 0.002). Conclusion. There are disease classification and SSc-related serum autoantibody differences between French and American patients with SSc. These differences help to explain variations in clinical features reported from different geographic regions. (First Release Nov 15 2006; J Rheumatol 2007;34:104–9) Key Indexing Terms:
SYSTEMIC SCLEROSIS From the Rheumatology Unit, Bichat Hospital, Paris, France; and Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA. Supported in part by a grant from the Société Française de Rhumatologie; the University of Pittsburgh Scleroderma Restricted Research Fund; REK Foundation, Austin, Texas; Arthritis Foundation, Western PA Chapter (Shoemaker Fund); and the Taub Laboratory Research Fund. O.C. Meyer, MD, Professor of Rheumatology; N. Somogyi, MD, Chef de Clinique, Rheumatology, University Paris VII; N. Fertig, MPH, Research Specialist; M. Lucas, MPH, Research Specialist; T.A. Medsger Jr, MD, Professor of Medicine, University of Pittsburgh. Address reprint requests to Prof. O.C. Meyer, Rheumatology Unit, Bichat Hospital, 46 rue H. Huchard, 75018 Paris, France. E-mail: olivier.meyer@bch.aphp.fr Accepted for publication September 20, 2006. |
