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
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    ABSTRACT: Sjögren's Syndrome (SS) is a debilitating autoimmune disease that primarily affects women. Patients with SS experience dry eyes and dry mouth in addition to systemic disease manifestations, including arthritis, peripheral neuropathy and pulmonary fibrosis. As in many autoimmune diseases, the inciting factors that precipitate SS are poorly understood. Patients with SS have periductal and perivascular lymphocytic infiltration of salivary and lacrimal tissue, and this is a hallmark of disease. While this infiltration is well characterized, the pathologic events that precede and cause this inflammatory cell recruitment are unknown. Although few studies have examined SS salivary tissue prior to disease onset, there is strong evidence for innate immune hyperactivity. Accordingly, processes such as apoptosis of glandular tissue, heightened inflammatory cytokine and chemokine production, and toll-like receptor (TLR) activation are described in early disease and are each linked to innate immune activation in murine models of disease and SS patients. This review will explore the relationship between innate immunity and SS pathogenesis prior to overt disease onset and discuss therapeutic strategies to mitigate disease progression in SS patients.
    Cytokine 03/2014; 67(2). DOI:10.1016/j.cyto.2014.02.009 · 2.87 Impact Factor
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    ABSTRACT: Objective. In this study we evaluated US as an additional classification item in the ACR classification of SS. Methods. Of 581 patients classified as either SS (n = 364) or non-SS (n = 217) based on the minimum requirements of the American-European Consensus Group (AECG) classification, 184 patients (102 SS and 82 non-SS) who had scored two or more positive or two or more negative results according to the ACR criteria were selected. The AECG classification was used as the gold standard. A parotid and/or submandibular gland that was assigned a score >= G1 was designated as SS positive. We evaluated US alone or with varying combinations of the ACR classification items in the diagnosis of SS. Results. The ACR criteria diagnosed the 184 patients with 91% sensitivity, 90% specificity and 91% accuracy. US alone diagnosed the 184 ACR patients with 79% sensitivity, 90% specificity and 83% accuracy, which was comparable to the results of US diagnosis in the AECG cohort (81%, 86% and 83%, respectively). Incorporating the US criteria as an alternative to one of the three ACR classification items achieved 89-91% sensitivity, 87-96% specificity and 89% or 92% accuracy, which was comparable to that of the original ACR classification. Furthermore, kappa analysis indicated that the results of the original ACR and US-replaced ACR classifications matched completely (kappa = 0.960-0.974). Conclusion. These results suggest that US can be used as an alternative to any of the three ACR classification items.
    Rheumatology (Oxford, England) 06/2014; 53(11). DOI:10.1093/rheumatology/keu238 · 4.44 Impact Factor
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    ABSTRACT: Sjogren's syndrome (SS) is a chronic autoimmune disease mainly affecting salivary and lacrimal glands. Current diagnostic criteria for SS utilize anti-Ro and anti-La as serological markers. Animal models for SS have identified novel autoantibodies, anti-salivary gland protein 1 (SP1), anti-carbonic anhydrase 6 (CA6) and parotid secretory protein (PSP). These novel antibodies are seen in the animals at an earlier stage of SS than anti-Ro and anti-La. The current studies were designed to evaluate these novel autoantibodies in the sera of well-characterized patients with dry eyes and dry mouth and lip biopsies from the Sjogren's International Collaborative Clinical Alliance (SICCA) to determine if they indeed identify SS with less severe disease than patients expressing anti-Ro and anti-La. Sera were obtained from SICCA registry in patients for whom lymphocytic foci per 4 mm(2) on the lip biopsies was either 0 (F = 0), <1 (F <1) or > 3 (F >3). ELISA assays were utilized to evaluate these sera for anti-Ro, anti-La, anti-SP1, anti-CA6, and anti-PSP. In patients with dry eyes and dry mouth but F = 0, increased expression of anti- CA6 was noted compared to the F <1 group (p = .032) or the F > 3 group (p = .006). Neither anti-PSP nor anti-SP1 reached statistical significance because of the small numbers in the F0 group, although there was a trend for their expression to be higher in the F0 group. On the other hand, the expression of anti-Ro was significantly reduced in the F0 group compared to the F <1 (p = .0021) and F > 3 (p = .0003) groups. The reduced expression of anti-La in the F0 group compared to the F <1 and F > 3 groups did not quite reach statistical significance. Anti-Ro and anti-La identify patients with SS and more severe disease than anti-SP1, anti-CA6, and anti-PSP. More studies are needed to identify the timing in the course of SS when these different autoantibodies are expressed and/or whether they are expressed in patients with different clinical manifestations.
    BMC Ophthalmology 04/2015; 15(1):38. DOI:10.1186/s12886-015-0023-1 · 1.08 Impact Factor

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