Clinical aspects
Primary Sjögren's syndrome in South Australia
S.A. Lyne1, S. Downie-Doyle2, S.E. Lester3, A. Quinlivan4, P. Toby Coates5, T.P. Gordon6, M. Rischmueller7
- Rheumatology Department, The Queen Elizabeth Hospital, Woodville South, and Flinders Medical School, Flinders University, Bedford Park, Australia.
- Rheumatology Department, The Queen Elizabeth Hospital, Woodville South, and Rheumatology Research Group, Basil Hetzel Institute, Woodville South, Australia.
- Rheumatology Department, The Queen Elizabeth Hospital, Woodville South, and Rheumatology Research Group, Basil Hetzel Institute, Woodville South, Australia.
- Rheumatology Department, The Queen Elizabeth Hospital, Woodville South, Australia.
- Adelaide Medical School, University of Adelaide and Central Northern Adelaide Renal and Transplantation Service, Adelaide, Australia.
- Flinders Medical School, Flinders University, Bedford Park; Immunology Department, Flinders Medical Centre, Bedford Park, and SA Pathology Immunology Department, Adelaide, Australia.
- Rheumatology Department, The Queen Elizabeth Hospital, Woodville South; Rheumatology Research Group, Basil Hetzel Institute, Woodville South, and Adelaide Medical School, University of Adelaide, Australia. maureen.rischmueller@sa.gov.au
CER13711
2020 Vol.38, N°4 ,Suppl.126
PI 0057, PF 0063
Clinical aspects
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PMID: 32940213 [PubMed]
Received: 20/06/2020
Accepted : 13/07/2020
In Press: 15/09/2020
Published: 22/10/2020
Abstract
OBJECTIVES:
To describe clinical and serological characteristics of a South Australian primary Sjögren’s syndrome (pSS) cohort.
METHODS:
The South Australian Sjögren’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.
CONCLUSIONS:
Despite the relatively small sample size of the South Australian cohort, clinical and serological characteristics correspond closely with international descriptions.