Serological and Immunogenetic Markers of Extraglandular Primary Sjögren's Syndrome

Abstract

The clinical course of 48 patients with primary Sjögren's syndrome (primary SS) was reviewed. Forty-three north European patients were typed for HLA class I and class II alloantigens. In this population with primary SS HLA B8, DR3 and DRw52 all occurred more frequently than in the control population (P < 0.009, P < 0.0035, P < 0.02 respectively). The subgroup of primary SS patients with antibodies to Ro and/or La antigen had the greatest prevalence of DR3 (relative risk 33.4). The primary SS patients fall into two distinct groups: those with extraglandular disease in whom lymphopaenia, hypergammaglobulinaemia, antibodies to Ro and/or La and HLA DR3 were all more frequent and those patients with either glandular disease alone or only one extraglandular feature. There was no difference in disease duration between the two groups, although on average the latter group were 10 years older.

Lack of association between mannose-binding lectin gene polymorphisms and primary Sjögren's syndrome

“…results were compared with findings in the control group (Table 1). Increasing antibody positivity reflects diversification of the autoimmune response to La/Ro RNP (10) and is associated with more severe glandular and extraglandular disease in primary SS (13,14). The control allele and haplotype frequencies were in Hardy-Weinberg equilibrium and were similar to previously published frequencies (12).…”

Prevalence and clinical relevance of 52-kDa and 60-kDa Ro/SS-A autoantibodies in Japanese patients with systemic sclerosis

“…Although we routinely try to screen for the presence of Sjögren’s syndrome, the patients who had clinical symptoms of Sjögren’s syndrome might have been likely to have further examinations. Finally, racial differences might be an important factor that would explain the differences, as anti-Ro/SS-A has been known to be related with specific human leucocyte antigens 22. However, there have been no other reports describing the prevalence of Sjögren’s syndrome in Asian patients with SSc.…”

Sjögren's Syndrome

“…Studies suggest a linkage of pSS with certain HLA alleles: HLA-B8, HLA-DR3, and DRW52, as well as HLA-DQA1 and HLA-DQB1 loci (Pease et al, 1993). Certain HLA-DR and DQ haplotypes are associated with PSS/autoantibody formation (Bolstad et al, 2001;Loiseau et al, 2001).…”