Primary Sjögren's syndrome as a systemic disease: A study of participants enrolled in an International Sjögren's syndrome registry

Abstract

Objective To study the prevalence of extra-glandular manifestations (EGM) in primary Sjögren’s Syndrome (pSS) among participants enrolled in the Sjögren’s International Collaborative Clinical Alliance (SICCA) registry. Methods 1927 participants in the SICCA registry were studied, including 886 participants who met the 2002 American-European consensus group (AECG) criteria for pSS, 830 “intermediate” cases who had some objective findings of pSS but did not meet AECG criteria, and 211 control individuals. We studied the prevalence of immunologic and hematologic laboratory abnormalities; specific rheumatologic examination findings; and physician confirmed thyroid, liver, kidney disease and lymphoma among SICCA participants. Results Laboratory abnormalities, including hematologic abnormalities, hypergammaglobulinemia and hypocomplementemia, frequently occurred among pSS cases, and were more common among the intermediate cases than among control participants. Cutaneous vasculitis and lymphadenopathy were also more common among pSS cases. In contrast, the frequency of physician confirmed diagnoses of thyroid, liver and kidney disease, and lymphoma was low and only primary biliary cirrhosis was associated with pSS cases status. Rheumatologic and neurologic symptoms were common among all SICCA participants, regardless of case status. Conclusions Data from the international SICCA registry support the systemic nature of pSS, manifest primarily in terms of specific immunologic and hematologic abnormalities. The occurrence of other systemic disorders among this cohort is relatively uncommon. Previously reported associations may be more specific to select patient subgroups, such as those referred for evaluation of certain neurologic, rheumatologic or other systemic manifestations.

The Potential Role for Early Biomarker Testing as Part of a Modern, Multidisciplinary Approach to Sjögren’s Syndrome Diagnosis

“…Data from the Sjögren's International Collaborative Clinical Alliance (SICCA) registry suggest that primary SS is characterized by immunologic and hematologic abnormalities that may affect multiple organ systems [14]. Patients with SS have an increased risk of cerebrovascular events and myocardial infarction [15], and are more than twice as likely as age-and sex-matched controls to have hypertension and hypertriglyceridemia.…”

Prevalence of Extraglandular Manifestations in Patients With Primary Sjögren’s Syndrome in Southern Norway: A Comparison to the Literature

“…21 There is a limitation of studies on EGM in pSS in the completely healthy (asymptomatic) control groups of individuals: "It is not feasible to assemble sufficiently large, populationbased control groups of completely healthy individuals". 22 Variations detected by Sjögren's International Collaborative Clinical Alliance Registry 22 in the actual populations, recruitment sources, investigation methods as well as the inclusion criteria and definitions regarding organ involvement utilized by the various investigators may, together with environmental factors, genetic, and race differences, be the causes for such differences. Furthermore, there is a relationship between the number of systemic autoantibodies and the number of EGM in pSS with a high prevalence of anti-Sjögren syndrome A antigen, anti-Sjögren syndrome B antigen, anti-nuclear antibodies, and immunoglobulin M-rheumatoid factor antibodies, indicating that a more disturbed immune system (as characterized by the presence of hypergammaglobulinemia and multiple autoantibodies formed by hyper-reactive B-cells) may result in more EGM.…”

Ocular Manifestations of Sjögren Syndrome

“…These criteria were subsequently reexamined in 2002, and their revised version, the American European Consensus Group (AECG) criteria set, has rapidly become the standard reference for SS [19]. However, due to its restrictive and stringent nature, new criteria have been proposed in 2012 based on the analysis of the Sjögren's International Collaborative Clinical Alliance (SICCA) cohort [20] and approved by the American College of Rheumatology (ACR) [21]. graft versus host disease; IgG4-related disease [23].…”