Abstract
The aim of the study was to define main symptoms of clinical appearance and immunoserological profile of male patients with primary Sjögren’s syndrome (pSS). Four hundred and ninety-two patients fulfilling the European–American Consensus Criteria for pSS were involved in this study. The mean age of the patients was 55.93 years (55.67 years in women and 56.18 years in men). The female–male ratio was 7:1 (432 and 60 patients, respectively). At the time of the diagnosis of pSS, glandular, extraglandular manifestations (EGMs), and immunoserological parameters were assessed. The major EGMs differ between genders. Arthritis was frequently presented as EGM in both genders, but the ratio was higher in men (68% vs. 42%). Various vasculitis symptoms and lymphadenopathy were more frequent in men than in women, in contrast to Raynaud’s phenomenon or autoimmune thyroiditis. Anti-SS-A and anti-SS-B were the most frequent autoantibodies in both genders, although autoantibodies against anti-nuclear factor and extractable nuclear antigens also presented in some patients. In a few cases, there were other specific autoantibodies correlated with EGMs, such as double-stranded DNA, anti-neutrophilic–cytoplasmic antibody, cyclic-citrullinated peptide, anti-thyreoglobuline antibodies, and anti-thyreoid-peroxidase antibodies. Based upon our large cohort of patients with pSS, we conclude that, although the disease is more frequent in women usually about climax, it develops also in men with the predominant symptoms of vasculitis or arthritis besides keratoconjunctivitis sicca or xerostomy.
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References
Manthorpe R (2002) Sjögren’s syndrome criteria. Ann Rheum Dis 61:482–484
Jonsson R, Moen K, Vestrheim D et al (2002) Current issues in Sjögren’s syndrome. Oral Dis 8:130–140
Márton K, Boros I, Varga G et al (2006) Evaluation of palatal saliva flow rate and oral manifestations in patients with Sjögren’s syndrome. Oral Dis 12:480–486
Csepregi A, Szodoray P, Zeher M (2002) Do autoantibodies predict autoimmune liver disease in primary Sjögren’s syndrome? Data of 180 patients upon a 5 year follow-up. Scand J Immunol 56:623–629
Szodoray P, Barta Z, Lakos G et al (2004) Coeliac disease in Sjögren’s syndrome—a study of 111 Hungarian patients. Rheumatol Int 24:278–282
Fox RI, Kang HI (1992) Pathogenesis of Sjögren’s syndrome. Rheum Dis Clin North Am 18:517–538
Mikulicz J (1888) Discussion at Verein fur wissenschaftliche Heilkunde zu königsberg. Berl Klin Wocchenschr 25:759
Parke AL (2000) Sjogren’s syndrome: a women’s health problem. J Rheumatol Suppl 61:4–5
Anaya JM, Lin GT, D’Souza E (1995) Primary Sjogren’s syndrome in men. Ann Rheum Dis 54:748–751
Drosos AA, Tsiakou EK (1997) Subgroups of primary Sjögren’s syndrome. Sjögren’s syndrome in male and paediatric Greek patients. Ann Rheum Dis 56:333–335
Brennan MT, Fox PC (1999) Sex differences in primary Sjogren’s syndrome. J Rheumatol 26:2373–2376
Molina R, Provost TT, Arnett FC (1986) Primary Sjogren’s syndrome in men. Clinical, serologic, and immunogenetic features. Am J Med 80:23–31
Yazawa Y, Okada K, Fukui J et al (1997) Clinical features of Sjögren syndrome. Nippon Jibiinkoka Gakkai Kaiho 100:429–435
Cervera R, Font J, Ramos-Casals M (2000) Primary Sjogren’s syndrome in men: clinical and immunological characteristics. Lupus 9:61–64
Diaz-Lopez C, Geli C, Corominas H (2004) Are there clinical or serological differences between male and female patients with primary Sjogren’s syndrome. J Rheumatol 31:1352–1355
Merrill JT, Dinu AR, Lahita RG (1996) Autoimmunity: the female connection. Medscape Womens Health 1:5
Ansar Ahmed S, Penhale WJ, Talal N (1985) Sex hormones, immune responses, and autoimmune diseases. Mechanisms of sex hormone action. Am J Pathol 121:531–551
Lahita RG (1996) The connective tissue diseases and the overall influence of gender. Int J Fertil Menopausal Stud 41:156–165
Lahita RG (1998) Collagen disease: the enemy within. Int J Fertil Womens Med 43:229–234
Rider V, Foster RT, Evans M et al (1998) Gender differences in autoimmune diseases: estrogen increases calcineurin expression in systemic lupus erythematosus. Clin Immunol Immunopathol 89:171–180
Cutolo M, Sulli A, Capellino S et al (2004) Sex hormones influence on the immune system: basic and clinical aspects in autoimmunity. Lupus 13:635–638
Porola P, Laine M, Virkki L et al (2007) The influence of sex steroids on Sjögren’s syndrome. Ann N Y Acad Sci 1108:426–432
Laine M, Porola P, Udby L et al (2007) Low salivary dehydroepiandrosterone and androgen-regulated cysteine-rich secretory protein 3 levels in Sjögren’s syndrome. Arthritis Rheum 56:2575–2584
Van Vollenhoven RF, McGuire JL (1994) Estrogen, progesterone, and testosterone: can they be used to treat autoimmune diseases. Cleve Clin J Med 61:276–284
Da Silva JA, Hall GM (1992) The effects of gender and sex hormones on outcome in rheumatoid arthritis. Baillieres Clin Rheumatol 6:196–219
Verthelyi D (2001) Sex hormones as immunomodulators in health and disease. Int Immunopharmacol 1:983–993
Beeson PB (1994) Age and sex associations of 40 autoimmune diseases. Am J Med 96:457–462
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This work was funded by The National Research Fund/Hungary/(OTKA: K60534 and K42631) and by the Bolyai János academic research fellowship for Peter Szodoray.
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Horvath, I.F., Szodoray, P. & Zeher, M. Primary Sjögren’s syndrome in men: clinical and immunological characteristic based on a large cohort of Hungarian patients. Clin Rheumatol 27, 1479–1483 (2008). https://doi.org/10.1007/s10067-008-0944-7
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DOI: https://doi.org/10.1007/s10067-008-0944-7