Primary Sjogren's syndrome in South Australia

Abstract

Objective: To describe clinical and serological characteristics of a South Australian primary Sjogren's syndrome (pSS) cohort. Methods: The South Australian Sjogren's Syndrome Research Clinic and Database is a clinical cohort of patients with pSS at a single site. Baseline clinical and laboratory data from 172 patients were retrospectively examined to determine their prevalence and clinical associations. Results were compared to findings from 10,500 patients from The Big Data Sjogren Project Consortium; an international, multicentre registry established in 2014, which included the South Australian data. Results: Of 172 South Australian patients with pSS, 90.1% were female with a mean age at diagnosis of 57 years. Ocular and oral sicca symptoms were common, affecting 97.1% and 99.4% respectively. Anti-Ro +/- La positivity was detected in 82.6%, ANA positivity in 77%, and in 9% of patients both ANA and ENA were negative. Mean ESSDAI was 6.8 at baseline, slightly higher than the international cohort at 6.1; the most commonly positive domains being biological, articular and glandular. Pulmonary manifestations represented the most significant morbidity over time. Lymphoma was recorded in 5.2% of patients and congenital heart block in 4 offspring of 52 patients with longitudinal follow-up (7.7%), although incomplete data likely resulted in underestimation of both. Conclusion: Despite the relatively small sample size of the South Australian cohort, clinical and serological characteristics correspond closely with international descriptions.