Is primary Sjögren's syndrome an orphan disease? A critical appraisal of prevalence studies in Europe: Table 1

Cornec, Divi; Chiche, Laurent

doi:10.1136/annrheumdis-2014-206860

Cited by 51 publications

(32 citation statements)

References 13 publications

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“…To accurately estimate the prevalence in Europe, we have to focus on robust studies using good methodology and large background population. To estimate the prevalence of pSS in Europe, Cornec and Chiche [10] appointed three population-based studies which had used the AECG classification criteria, which were based on large background populations, and which had used effective case-finding methods [11 12•, 13•]. A prevalence of 1–9 cases per 10,000 people was found, which gave a combined prevalence of nearly 39/100,000 (~0.04 %), which is far lower than previously reported (up to 6 %) [10].…”

Section: Epidemiology Of Sjögren’s Syndromementioning

confidence: 99%

Epidemiology of Sjögren’s Syndrome—from an Oral Perspective

Bolstad

Skarstein

2016

Curr Oral Health Rep

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Oral symptoms are among the most distressing manifestations for patients with Sjögren’s syndrome (SS). The feeling of dry mouth is unpleasant, and hyposalivation may contribute to difficulty in speaking, chewing and swallowing and reduced quality of life. Reduced salivary flow increases the risk for dental caries and problems with prosthetic replacement. It seems that SS is not as frequently occurring as previously anticipated. Population-based prevalence studies on primary SS in Europe, conducted on large background populations and in accordance with the AECG criteria, reported of a prevalence of 1–9 cases per 10,000 people. This gives a combined prevalence of nearly 39/100,000 (~0.04 %). The cause of Sjögren’s syndrome is even now not fully understood, and the treatment of oral symptoms is still mostly palliative. Hopefully, useful information will appear from the new methods that are now available for genome wide association studies, epigenetics, DNA methylation studies, and proteomics. Similarly, this is anticipated for the immunological side of the story. The interferon signature, the interferon γ/interferon α mRNA ratio, and CXCL13 are among the proposed biomarkers of active disease. In this review, we provide an update on oral aspects of Sjögren’s syndrome with emphasis on the latest publications on these topics.

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Section: Epidemiology Of Sjögren’s Syndromementioning

confidence: 99%

Epidemiology of Sjögren’s Syndrome—from an Oral Perspective

Bolstad

Skarstein

2016

Curr Oral Health Rep

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“…Previous epidemiological studies have reported extraordinarily discrepant prevalence figures for pSS, ranging from 0.01% of the general population to more than 3%, which has generated a debate as to whether pSS should be considered as a rare or frequent disease (3). …”

Section: Introductionmentioning

confidence: 99%

Prevalence of Primary Sjögren's Syndrome in a US Population‐Based Cohort

Gabriel

Crowson

Matteson

et al. 2017

Arthritis Care & Research

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110

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Objective To report the point prevalence of primary Sjögren’s syndrome (pSS) in the first population-based study performed in the United States. Methods Cases of all potential pSS patients living in Olmsted County, Minnesota on January 1, 2015 were retrieved using the Rochester Epidemiology Project resources, and ascertained by manual medical record review. pSS cases were defined according to physician diagnosis. The use of diagnostic tests was assessed and the performance of classification criteria was evaluated. The number of prevalent cases in 2015 was also projected based on 1976–2005 incidence data from the same source population. Results A total of 106 patients with pSS were included in the study: 86% were female, with a mean (SD) age of 64.6 (15.2) years and disease duration of 10.5 (8.4) years. A majority were anti-SSA positive (75%) and/or anti-SSB positive (58%), but only 22% met American-European Consensus Group or American College of Rheumatology criteria because the other tests required for disease classification were rarely performed in clinical practice (ocular dryness objective assessment, salivary gland functional or morphologic tests, or salivary gland biopsy). According to the physician diagnosis, age and sex adjusted prevalence of pSS was 10.3/10,000 inhabitants, but according to classification criteria this prevalence was only 2.2/10,000. The analysis based on previous incidence data projected a similar 2015 prevalence rate of 11.0/10,000. Conclusion The prevalence of pSS in this geographically well-defined population was estimated between 2 and 10/10,000 inhabitants. Physicians rarely used tests included in the classification criteria to diagnose the disease in this community setting.

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“…The epidemiology of pSS is poorly defined 22, 23 . We recently reported that the 2015 prevalence of pSS in Olmsted County was 10.3/10,000 inhabitants according to the physician diagnosis, but only 2.2/10,000 according to classification criteria 24 .…”

Section: Introductionmentioning

confidence: 99%

Incidence and Mortality of Physician-Diagnosed Primary Sjögren Syndrome

Gabriel

Crowson

Matteson

et al. 2017

Mayo Clinic Proceedings

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Objective To estimate the incidence and mortality rates of physician-diagnosed primary Sjögren’s syndrome (pSS) among residents of Olmsted County, Minnesota, and their evolution over time. Patients and Methods All medical records of patients with a diagnosis or suspicion of SS in Olmsted County, MN, from January 1, 2006 to December 31, 2015 were reviewed to identify incident cases of pSS (defined according to physician diagnosis). These cases were combined with a previous 1976–2005 incident cohort from the same population. Incidence rates were age and sex adjusted to the US white 2010 population. Survival rates were compared with the expected rates in the population of Minnesota. Results With 61 incident cases of pSS diagnosed in Olmsted County in 2006–2015, the total cohort included 172 patients with incident pSS in 1976–2015. Of the 172 patients, 151 (88%) were women and 161 (94%) were white, with a mean (SD) age at diagnosis of 58.3 (16.7) years. The average age- and sex- adjusted annual incidence for 2006–2015 was 5.9 per 100,000 population (95%CI 4.4–7.4), and overall incidence for the entire period was 5.8 (95% CI: 4.9–6.6) per 100,000. The incidence increased with calendar time over the 40-year period (P=.005). There was no difference in mortality in the pSS cohort compared to expected (standardized mortality ratio 1.15, 95%CI 0.86–1.50). Conclusions The average annual incidence of pSS in this population based-cohort was 5.8/100,000, with a progressive increase over the 40 years covered by the study. Overall survival of pSS patients was not different from the general population.

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Is primary Sjögren's syndrome an orphan disease? A critical appraisal of prevalence studies in Europe: Table 1

Cited by 51 publications

References 13 publications

Epidemiology of Sjögren’s Syndrome—from an Oral Perspective

Epidemiology of Sjögren’s Syndrome—from an Oral Perspective

Prevalence of Primary Sjögren's Syndrome in a US Population‐Based Cohort

Incidence and Mortality of Physician-Diagnosed Primary Sjögren Syndrome

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