Rheumatology Advance Access published online on March 23, 2007
Rheumatology, doi:10.1093/rheumatology/kem021
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Systemic sclerosis–rheumatoid arthritis overlap syndrome: a unique combination of features suggests a distinct genetic, serological and clinical entity
Third Department of Medicine, 1Rheumatology Division, and 2Laboratory of Immunology, University of Debrecen Medical and Health Science Centre, Debrecen, H-4004, 3Research Group of Autoimmune Diseases, Hungarian Academy of Sciences, Debrecen, H-4004 and 4Department of Immunology and Rheumatology, Hungarian Brothers of St. John of God and University of Pécs, Pécs, H-7642, Hungary
Correspondence to:
Gabriella Szücs, Third Department of Medicine, Rheumatology Division, University of Debrecen Medical and Health Science Center, 22 Móricz Zs str Debrecen H-4004, Hungary. E-mail: szucsg{at}iiibel.dote.hu
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Abstract |
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Objective. To determine the genetic, clinical and serological characteristics of systemic sclerosis (SSc)-rheumatoid arthritis (RA) overlap syndrome.
Methods. Clinical manifestations and immunolaboratory features of 22 SSc-RA patients were assessed. The HLA-DR genotype of the 22 SSc-RA patients determined by SSP-PCR was compared with that of 38 SSc patients, 100 RA patients and 50 healthy controls.
Results. All overlap patients fulfilled the American College of Rheumatology (ACR) criteria for SSc and RA. Five of the 22 patients (23%) had diffuse cutaneous SSc (dcSSc) and 17 patients (77%) had limited cutaneous SSc (lcSSc). Antinuclear antibody, anti-Scl70, IgM rheumatoid factor and anti-CCP antibody positivity were detected in 22 (100%), 5 (23%), 16 (73%) and 18 patients (82%), respectively. Seventeen patients (77%) had pulmonary fibrosis, 12 (55%) had oesophageal dismotility, 11 (50%) had cardiac and five (23%) had renal involvement. Hand joint destruction was observed in 18 patients (82%). Significantly increased frequencies of HLA-DR3 (36% vs 5%), HLA-DR7 (9% vs 4%), HLA-DR11 (36% vs 7%) and HLA-DRw53 (23% vs 5%) were observed in SSc-RA compared with RA patients (P < 0.05). Allele frequencies of the ‘shared epitope’ (HLA-DR1 and -DR4) were significantly increased in SSc-RA (32% and 27%, respectively) and RA patients (46% and 31%, respectively) in comparison with SSc patients (10.5% and 16%, respectively) or healthy controls (16% and 14%, respectively) (P < 0.05).
Conclusions. To date this is the largest SSc-RA overlap cohort. Genetics, clinical and immunolaboratory features suggest a mixed phenotype. Our data suggest that SSc-RA overlap syndrome may be a distinct genetic, immunological and clinical entity.
KEY WORDS: Rheumatoid arthritis, Systemic sclerosis, Overlap syndrome, HLA-DR
* Co-first authors: equal contribution.
Submitted 5 July 2006;
revised version accepted 12 January 2007.
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