Article

An early view of the international Sjögren's syndrome registry

Oral Pathology, University of California at San Francisco, Box 0422, San Francisco, California 94143, USA.
Arthritis & Rheumatology (Impact Factor: 7.87). 05/2009; 61(5):711-4. DOI: 10.1002/art.24397
Source: PubMed

ABSTRACT In the process of developing new classification criteria for SS, we use area-proportional Venn diagrams to visualize the interrelationship between various phenotypic characteristics of SS (14). Figure 1 illustrates the overlapping relationships of 3 objective SS signs in the cohort (72% exhibit positive ocular staining for KCS, 42% positive LSG biopsy specimen, 39% positive serum anti-SSA and/or anti-SSB, and 20% none of these). Interestingly, 3% of the participants had only an abnormal focus score, 2% had only a positive serology to anti-SSA or anti-SSB, whereas 28% had only an abnormal ocular staining score. The high proportion of participants with only abnormal ocular staining led us to compare this subgroup (termed KCS-only; n = 323) with those having KCS and at least 1 of the 2 other main phenotypic features of SS, specifically focal lymphocytic sialadenitis with a focus score >1 and/or anti-SSA or anti-SSB antibodies (termed SS-KCS; n = 510). KCS-only participants had significantly lower levels of serum autoantibodies (RF, ANA titer, IgG), better ocular physiologic tests (Schirmer's test, tear break-up time), more frequent use of anticholinergic drugs and/or cigarettes (P < 0.0001 for all), and were older (P = 0.001) compared with SS-KCS participants. This suggests that KCS-only cases may represent a distinct entity of seronegative KCS that is significantly less severe than SS-KCS. Additional recall data will allow us to explore if KCS-only subjects progress into SS-KCS or whether KCS-only is a disease entity distinct from SS.

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    ABSTRACT: According to the classification criteria of the American-European Consensus Group (AECG), the prevalence of primary Sjögren's syndrome (pSS) of about 0.2% in the adult population and a yearly incidence of 4/100.000 in the general population are far lower than previously assumed. Moreover, the repeatedly reported male/female ratio of 1:9 seems to lie more in the range of 1:20. Male pSS patients show fewer immunological, histopathological or sialographic findings and organ involvement. Information on age at disease onset has also changed over the last decade. Recent studies indicate an onset age of approximately 45 years as compared to 56 in earlier studies of the last decade. Patients with an early disease onset are more frequently positive for rheumatoid factor (RF) and/or anti-Ro/SS-A. These patients also seem to have a higher risk of developing hypocomplementemia or lymphadenopathy. As compared to earlier cohorts, the introduction of the rather specific AECG criteria will probably result in the participation of fewer men, younger patients in general and of more seriously ill patients in future cohorts. The change in the spectrum of pSS patients obviously reflects the altered classification criteria since the AECG criteria require anti-Ro/La positivity and therefore exclude a high number of patients with other immunological markers who also show severe sicca symptoms and organ involvements. About 5%-10% of pSS patients in rheumatological care suffer from severe extraglandular manifestations, which generally occur soon after disease onset. In particular, palpable purpura, hypocomplementemia, cryoglobulinemia and lymphoma are associated with increased mortality. In Germany, approximately one tenth of Sjögren syndrome patients receive specialized rheumatological care. There is still insufficient knowledge about the vast majority of pSS patients who are not treated by rheumatologists. These patients, as well as all those who, according to the AECG criteria, are not classified as having pSS either due to anti-Ro/La negativity or having secondary Sjögren's syndrome, probably add up to at least 0.4% of the adult population which, at present, suffers from considerable immunopathologic sicca symptoms.
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