Advertisement
Mayo Clinic Proceedings Home

Incidence of Physician-Diagnosed Primary Sjögren Syndrome in Residents of Olmsted County, Minnesota

      Objectives

      To estimate the incidence of physician-diagnosed primary Sjögren syndrome (SS) among residents of Olmsted County, Minnesota, in the setting of usual medical care and to determine how often objective criteria are available in the medical records of such patients.

      Patients and Methods

      We reviewed all medical records of residents in Olmsted County with physician-diagnosed SS from 1976 to 1992 to determine whether they had undergone objective tests for keratoconjunctivitis sicca, salivary dysfunction, or serologic abnormality. Confounding illnesses were excluded. To identify misclassified cases, all records from patients with xerostomia or kerato-conjunctivitis sicca were also reviewed. The average annual SS incidence rates were calculated by considering the entire population to be at risk.

      Results

      Of 75 patients with onset of SS during the study period, 53 had primary SS. All patients were white, 51 (96.2%) were women, and the mean ± SD age was 59±15.8 years. The age- and sex-adjusted annual incidence was 3.9 per 100,000 population (95% confidence interval, 2.8–4.9) for patients with primary SS. Eleven patients (20.8%) with physician-diagnosed SS had no documentation of objective eye, mouth, or laboratory abnormalities. Objective evaluations performed most frequently were laboratory and ocular tests and least often were investigations of xerostomia.

      Conclusions

      The average annual incidence rate for physician-diagnosed primary SS in Olmsted County is about 4 cases per 100,000 population. These data probably underestimate the true incidence because they are based on usual medical care of patients with SS in a community setting, rather than on a case-detection survey. In the future, a true incidence may be possible with a higher index of suspicion, greater attention to objective tests, and increased awareness of new classification criteria for SS. For epidemiological studies based on existing data, application of current criteria may not be feasible, and consensus on criteria for such studies would be useful.
      ANA (antinuclear antibody), CI (confidence interval), HIV (human immunodeficiency virus), KCS (keratoconjunctivitis sicca), RA (rheumatoid arthritis), SS (Sjögren syndrome), SLE (systemic lupus erythematosus), XS (xerostomia)
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Mayo Clinic Proceedings
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      REFERENCES

        • Shearn MA
        Sjögren's syndrome.
        Med Clin North Am. 1977; 61: 271-282
        • Anderson LG
        • Talal N
        The spectrum of benign to malignant lymphoproliferation in Sjögren's syndrome.
        Clin Exp Immunol. 1972; 10: 199-221
        • Bloch KJ
        • Buchanan WW
        • Wohl MJ
        • Bunim JJ
        Sjögren's syndrome: a clinical, pathological, and serological study of sixty-two cases.
        Medicine (Baltimore). 1965; 44: 187-231
        • Drosos AA
        • Andonopoulos AP
        • Costopoulos JS
        • Papadimitriou CS
        • Moutsopoulos HM
        Prevalence of primary Sjögren's syndrome in an elderly population.
        Br J Rheumatol. 1988; 27: 123-127
        • Whaley K
        • Williamson J
        • Wilson T
        • et al.
        Sjögren's syndrome and autoimmunity in a geriatric population.
        Age Ageing. 1972; 1: 197-206
        • Strickland RW
        • Tesar JT
        • Berne BH
        • Hobbs BR
        • Lewis DM
        • Welton RC
        The frequency of sicca syndrome in an elderly female population.
        J Rheumatol. 1987; 14: 766-771
        • Sjögren H
        Zur Kenntnis der Keratoconjunctivitis sicca (Keratitis Filiformis bei Hypofunktion der Tränendrüsen).
        Acta Ophthalmol (Copenh). 1933; 11: 1-151
        • Jacobsson LT
        • Axell TE
        • Hansen BU
        • et al.
        Dry eyes or mouth—an epidemiological study in Swedish adults, with special reference to primary Sjögren's syndrome.
        J Autoimmun. 1989; 2: 521-527
        • Zhang NZ
        • Shi CS
        • Yao QP
        • et al.
        Prevalence of primary Sjögren's syndrome in China.
        J Rheumatol. 1995; 22: 659-661
        • Tomsic M
        • Logar D
        • Grmek M
        • Perkovic T
        • Kveder T
        Prevalence of Sjögren's syndrome in Slovenia.
        Rheumatology (Oxford). 1999; 38: 164-170
        • Thomas E
        • Hay EM
        • Hajeer A
        • Silman AJ
        Sjögren's syndrome: a community-based study of prevalence and impact.
        Br J Rheumatol. 1998; 37: 1069-1076
        • Manthorpe R
        • Oxholm P
        • Prause JU
        • Schiodt M
        The Copenhagen criteria for Sjögren's syndrome.
        Scand J Rheumatol Suppl. 1986; 61: 19-21
        • Skopouli FN
        • Drosos AA
        • Papaioannou T
        • Moutsopoulos HM
        Preliminary diagnostic criteria for Sjögren's syndrome.
        Scand J Rheumatol Suppl. 1986; 61: 22-25
        • Homma M
        • Tojo T
        • Akizuki M
        • Yamagata H
        Criteria for Sjögren's syndrome in Japan.
        Scand J Rheumatol Suppl. 1986; 61: 26-27
        • Fox RI
        • Robinson CA
        • Curd JG
        • Kozin F
        • Howell FV
        Sjögren's syndrome: proposed criteria for classification.
        Arthritis Rheum. 1986; 29: 577-585
        • Vitali C
        • Bombardieri S
        • Moutsopoulos HM
        • et al.
        Preliminary criteria for the classification of Sjögren's syndrome: results of a prospective concerted action supported by the European Community.
        Arthritis Rheum. 1993; 36: 340-347
        • Fox RI
        • Saito I
        Criteria for diagnosis of Sjögren's syndrome.
        Rheum Dis Clin North Am. 1994; 20: 391-407
        • Melton III, LJ
        History of the Rochester Epidemiology Project.
        Mayo Clin Proc. 1996; 71: 266-274
        • Bergstralh EJ
        • Offord KP
        • Chu C-P
        • Beard CM
        • O'Fallon WM
        • Melton III, LJ
        Calculating incidence, prevalence and mortality rates for Olmsted County, Minnesota residents: an update. Technical Report Series, No. 49. Section of Biostatistics, Mayo Clinic, Rochester, MinnMarch 1992
        • Kurland LT
        • Hauser WA
        • Ferguson RH
        • Holley KE
        Epidemiologic features of diffuse connective tissue disorders in Rochester, Minn, 1951 through 1967, with special reference to systemic lupus erythematosus.
        Mayo Clin Proc. 1969; 44: 649-663
        • Michet Jr, CJ
        • McKenna CH
        • Elveback LR
        • Kaslow RA
        • Kurland LT
        Epidemiology of systemic lupus erythematosus and other connective tissue diseases in Rochester, Minnesota, 1950 through 1979.
        Mayo Clin Proc. 1985; 60: 105-113
        • Hochberg MC
        The incidence of systemic lupus erythematosus in Baltimore, Maryland, 1970–1977.
        Arthritis Rheum. 1985; 28: 80-86
        • Jonsson H
        • Nived O
        • Sturfelt G
        • Silman A
        Estimating the incidence of systemic lupus erythematosus in a defined population using multiple sources of retrieval.
        Br J Rheumatol. 1990; 29: 185-188
        • Gudmundsson S
        • Steinsson K
        Systemic lupus erythematosus in Iceland 1975 through 1984: a nationwide epidemiological study in an unselected population.
        J Rheumatol. 1990; 17: 1162-1167
        • McCarty DJ
        • Manzi S
        • Medsger Jr, TA
        • Ramsey-Goldman R
        • Laporte RE
        • Kwoh CK
        Incidence of systemic lupus erythematosus: race and gender differences.
        Arthritis Rheum. 1995; 38: 1260-1270
        • Johnson AE
        • Gordon C
        • Palmer RG
        • Bacon PA
        The prevalence and incidence of systemic lupus erythematosus in Birmingham, England: relationship to ethnicity and country of birth.
        Arthritis Rheum. 1995; 38: 551-558
        • Linos A
        • Worthington JW
        • O'Fallon WM
        • Kurland LT
        The epidemiology of rheumatoid arthritis in Rochester, Minnesota: a study of incidence, prevalence, and mortality.
        Am J Epidemiol. 1980; 111: 87-98
        • Atkinson JC
        • Wu AJ
        Salivary gland dysfunction: causes, symptoms, treatment.
        J Am Dent Assoc. 1994; 125: 409-416
        • Billings RJ
        • Proskin HM
        • Moss ME
        Xerostomia and associated factors in a community-dwelling adult population.
        Community Dent Oral Epidemiol. 1996; 24: 312-316
        • Nederfors T
        Xerostomia: prevalence and pharmacotherapy: with special reference to beta-adrenoceptor antagonists.
        Swed Dent J Suppl. 1996; 116: 1-70
        • Thomson WM
        • Brown RH
        • Williams SM
        Medication and perception of dry mouth in a population of institutionalised elderly people.
        N Z Med J. 1993; 106: 219-221
        • Locker D
        Subjective reports of oral dryness in an older adult population.
        Community Dent Oral Epidemiol. 1993; 21: 165-168
        • Osterberg T
        • Birkhed D
        • Johansson C
        • Svanborg A
        Longitudinal study of stimulated whole saliva in an elderly population.
        Scand J Dent Res. 1992; 100: 340-345
        • O'Grady NP
        Incidence of dry mouth complaint in Cork Dental Hospital patient population.
        Stoma (Lisb). August-October 1990; 2 (58.): 55-56
        • Chisholm DM
        • Waterhouse JP
        • Mason DK
        Lymphocytic sialadenitis in the major and minor glands: a correlation in postmortem subjects.
        J Clin Pathol. 1970; 23: 690-694
        • Damato BE
        • Allan D
        • Murray SB
        • Lee WR
        Senile atrophy of the human lacrimal gland: the contribution of chronic inflammatory disease.
        Br J Ophthalmol. 1984; 68: 674-680
        • Tomer Y
        • Shoenfeld Y
        Ageing and autoantibodies.
        Autoimmunity. 1988; 1: 141-149
        • Jacobsson L
        • Hansen BU
        • Manthorpe R
        • Hardgrave K
        • Neas B
        • Harley JB
        Association of dry eyes and dry mouth with anti-Ro/SS-A and anti-La/SS-B autoantibodies in normal adults.
        Arthritis Rheum. 1992; 35: 1492-1501
        • Vitali C
        • Bombardieri S
        • Moutsopoulos HM
        • European Study Group on Diagnostic Criteria for Sjögren's Syndrome
        • et al.
        Assessment of the European classification criteria for Sjögren's syndrome in a series of clinically defined cases: results of a prospective multicentre study.
        Ann Rheum Dis. 1996; 55: 116-121
        • Sibilia J
        Ro(SS-A) and anti-Ro(SS-A): an update.
        Rev Rhum Engl Ed. 1998; 65: 45-57
        • McQuillan GM
        • Coleman PJ
        • Kruszon-Moran D
        • Moyer LA
        • Lambert SB
        • Margolis HS
        Prevalence of hepatitis B virus infection in the United States: the National Health and Nutrition Examination Surveys, 1976 through 1994.
        Am J Public Health. 1999; 89: 14-18
        • Feng X
        Hepatitis C infection: a review.
        Lippincotts Prim Care Pract. 1999; 3: 345-353