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. 1995 Sep;54(9):748–751. doi: 10.1136/ard.54.9.748

Primary Sjögren's syndrome in men.

J M Anaya 1, G T Liu 1, E D'Souza 1, N Ogawa 1, X Luan 1, N Talal 1
PMCID: PMC1009992  PMID: 7495348

Abstract

OBJECTIVE--To describe the clinical expression of primary Sjögren's syndrome (SS) in men, focusing on extraglandular manifestations (EGM) and serological markers of disease. METHODS--In a cross sectional and comparative study, adult men with primary SS were identified from a cohort study on SS, and 26 age matched adult women with primary SS were selected as a control group. All patients met the European classification criteria for SS. They were compared for demographic, clinical and laboratory findings. RESULTS--Thirteen men with primary SS were identified. Mean age at onset was 39 (SEM 4) years and mean duration of disease was 7.8 (1) years. Sicca complex or parotitis was the presenting feature in eight patients (61.5%), and an EGM in five (38.5%). During the course of the disease, EGM were present in 12 patients (92%), polyarthralgias and lymphopenia being the most frequent (38.5% each). Rheumatoid factor was positive in 73% of patients, antinuclear antibodies in 85%, anti-(SS-A) in 62%, and anti-(SS-B) in 46%. No statistical differences in the frequency of EGM or in the presence of autoantibodies were observed between men and women. However, men patients were more likely to have EGM. CONCLUSION--Primary SS in men is an uncommon condition with clinical and serological characteristics similar to those observed in women. Sex hormones may be incriminated in the pathogenesis of SS. However, it remains poorly understood whether sex hormones play a major role in the severity of disease and have any importance with regard to treatment.

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Selected References

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  1. Alexander E. L., Arnett F. C., Provost T. T., Stevens M. B. Sjögren's syndrome: association of anti-Ro(SS-A) antibodies with vasculitis, hematologic abnormalities, and serologic hyperreactivity. Ann Intern Med. 1983 Feb;98(2):155–159. doi: 10.7326/0003-4819-98-2-155. [DOI] [PubMed] [Google Scholar]
  2. Ansar Ahmed S., Penhale W. J., Talal N. Sex hormones, immune responses, and autoimmune diseases. Mechanisms of sex hormone action. Am J Pathol. 1985 Dec;121(3):531–551. [PMC free article] [PubMed] [Google Scholar]
  3. Aziz K. E., McCluskey P. J., Wakefield D. Phenotypic and functional abnormalities in the peripheral blood T-cells of patients with primary Sjogren's syndrome. Cytometry. 1994 Mar 15;18(1):35–41. doi: 10.1002/cyto.990180108. [DOI] [PubMed] [Google Scholar]
  4. Gardiner P., Ward C., Allison A., Ashcroft T., Simpson W., Walters H., Kelly C. Pleuropulmonary abnormalities in primary Sjögren's syndrome. J Rheumatol. 1993 May;20(5):831–837. [PubMed] [Google Scholar]
  5. King P. D., McMurray R. W., Becherer P. R. Sjögren's syndrome without mixed cryoglobulinemia is not associated with hepatitis C virus infection. Am J Gastroenterol. 1994 Jul;89(7):1047–1050. [PubMed] [Google Scholar]
  6. Kraus A., Caballero-Uribe C., Jakez J., Villa A. R., Alarcón-Segovia D. Raynaud's phenomenon in primary Sjögren's syndrome. Association with other extraglandular manifestations. J Rheumatol. 1992 Oct;19(10):1572–1574. [PubMed] [Google Scholar]
  7. Molina R., Provost T. T., Arnett F. C., Bias W. B., Hochberg M. C., Wilson R. W., Alexander E. L. Primary Sjögren's syndrome in men. Clinical, serologic, and immunogenetic features. Am J Med. 1986 Jan;80(1):23–31. doi: 10.1016/0002-9343(86)90044-6. [DOI] [PubMed] [Google Scholar]
  8. Moll J. W., Markusse H. M., Pijnenburg J. J., Vecht C. J., Henzen-Logmans S. C. Antineuronal antibodies in patients with neurologic complications of primary Sjögren's syndrome. Neurology. 1993 Dec;43(12):2574–2581. doi: 10.1212/wnl.43.12.2574. [DOI] [PubMed] [Google Scholar]
  9. Moutsopoulos H. M. Sjögren's syndrome: autoimmune epithelitis. Clin Immunol Immunopathol. 1994 Aug;72(2):162–165. doi: 10.1006/clin.1994.1123. [DOI] [PubMed] [Google Scholar]
  10. Ramakrishna R., Chaudhuri K., Sturgess A., Manoharan A. Haematological manifestations of primary Sjögren's syndrome: a clinicopathological study. Q J Med. 1992 Jul;83(303):547–554. [PubMed] [Google Scholar]
  11. Skopouli F. N., Barbatis C., Moutsopoulos H. M. Liver involvement in primary Sjögren's syndrome. Br J Rheumatol. 1994 Aug;33(8):745–748. doi: 10.1093/rheumatology/33.8.745. [DOI] [PubMed] [Google Scholar]
  12. Starkebaum G., Dancey J. T., Arend W. P. Chronic neutropenia: possible association with Sjögren's syndrome. J Rheumatol. 1981 Jul-Aug;8(4):679–684. [PubMed] [Google Scholar]
  13. Talal N., Aufdemorte T. B., Kincaid W. L., Sayers B. S., Lynn J. T., 3rd Two patients illustrating lymphoma transition and response to therapy in Sjögren's syndrome. J Autoimmun. 1988 Apr;1(2):171–184. doi: 10.1016/0896-8411(88)90024-8. [DOI] [PubMed] [Google Scholar]
  14. Vitali C., Bombardieri S., Moutsopoulos H. M., Balestrieri G., Bencivelli W., Bernstein R. M., Bjerrum K. B., Braga S., Coll J., de Vita S. Preliminary criteria for the classification of Sjögren's syndrome. Results of a prospective concerted action supported by the European Community. Arthritis Rheum. 1993 Mar;36(3):340–347. doi: 10.1002/art.1780360309. [DOI] [PubMed] [Google Scholar]

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