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Incidence and Mortality of Physician-Diagnosed Primary Sjögren Syndrome

Time Trends Over a 40-Year Period in a Population-Based US Cohort
  • Gabriel Maciel
    Affiliations
    Division of Rheumatology, Mayo Clinic College of Medicine and Science, Rochester, MN

    Hospital Maciel, Montevideo, Uruguay
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  • Cynthia S. Crowson
    Affiliations
    Division of Rheumatology, Mayo Clinic College of Medicine and Science, Rochester, MN

    Division of Biomedical Statistics and Informatics, Department of Health Sciences Research, Mayo Clinic College of Medicine and Science, Rochester, MN
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  • Eric L. Matteson
    Correspondence
    Correspondence: Address to Eric L. Matteson, MD, MPH, Divisions of Rheumatology and Epidemiology, Mayo Clinic College of Medicine and Science, 200 First St SW, Rochester, MN 55905.
    Affiliations
    Division of Rheumatology, Mayo Clinic College of Medicine and Science, Rochester, MN

    Division of Epidemiology, Department of Health Sciences Research, Mayo Clinic College of Medicine and Science, Rochester, MN
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  • Divi Cornec
    Affiliations
    Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine and Science, Rochester, MN

    Rheumatology Department, Brest Teaching Hospital, Brest, France
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      Abstract

      Objective

      To estimate the incidence and mortality rates, and their evolution over time, of physician-diagnosed primary Sjögren syndrome (pSS) in residents of Olmsted County, Minnesota.

      Patients and Methods

      Medical records of patients with a diagnosis or suspicion of SS in Olmsted County from January 1, 2006, through December 31, 2015, were reviewed to identify incident cases of pSS (defined by physician diagnosis). These cases were combined with those from a 1976 through 2005 incident cohort (n=111) from the same population. Incidence rates were age and sex adjusted to the 2010 US white 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 from 2006 through 2015, the total cohort included 172 patients with incident pSS from 1976 through 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 through 2015 was 5.9 per 100,000 population (95% CI, 4.4-7.4 per 100,000 population), and the overall incidence for the entire period was 5.8 per 100,000 (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 with expected (standardized mortality ratio, 1.15; 95% CI, 0.86-1.50).

      Conclusion

      The average annual incidence of pSS in this population-based cohort was 5.8 per 100,000, with a progressive increase over the 40 years of the study. Overall survival of patients with pSS was not different from that of the general population.

      Abbreviations and Acronyms:

      ACR (American College of Rheumatology), AECG (American-European Consensus Group), ESSDAI (EULAR Sjögren syndrome Disease Activity Index), EULAR (European League Against Rheumatism), pSS (primary Sjögren syndrome)
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