Summary
Our case report describes a patient with Klinefelter's syndrome (KFS) associated with rheumatoid arthritis (RA). He had active RA in 1985 but his arthritis almost subsided in 1993 without intensive treatments for RA as well as KFS. Recently, the lower levels of testosterone in male RA patients, especially at the active phase has been reported. However, it is still questionable whether hypogonadism is a predisposing factor or just a consequence of disease. Since our case had a mild clinical course, and since the incidence of RA associated with KFS is very rare in comparison with other rheumatic diseases, may suggest that the low levels of testosterone are not a predisposing factor to the activity of RA.
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References
Ortie-Neu, C., LeRoy, E.C. The coincidence of Klinefelter's syndrome and systemic lupus erythematosus. Arthritis Rheum 1969, 12, 241–246.
Lahita, R.G., Bradlow, H.L. Klinefelter's syndrome: hormone metabolism in hypogonadal males with systemic lupus erythematosus. J Rheumatol 1987, (suppl 13) 14, 154–157.
Roubinian, J.R., Talal, N., Greenspan, J.S., Goodman, J.R., Siiteri, P.K. Effect of castration and sex hormone treatment survival, antinucleic acid antibodies and glomerulonephritis in NZB CX NZB F1 mice. J Exp Med 1978, 147, 1568–1583.
Deighton, C.M., Watson, M.J., Walker, D.J. Sex hormones in post-menopausal HLA-identical rheumatoid arthritis discordant sibling pairs. J Rheumatol 1992, 19, 1663–1667.
Gordon, D., Beastall, G.H., Thompson, J.A., Sturrock, R.D. Prolonged hypogonadism in male patients with rheumatoid arthritis during flares in disease activity. Br J Rheumatol 1988, 27, 440–444.
MacSween, R.N.M. Reticulum cell sarcoma and rheumatoid arthritis in patient with XY/XXY/XXXY Klinefelter's syndrome and normal intelligence. Lancet 1965, i, 460–461.
Tsung, S.H., Heckman, M.G. Klinefelter's syndrome, immunological disorders, and malignant neoplasm. Arch Pathol 1974, 98, 351–354.
Ansar Ahmed, S., Talal, N. Sex hormones and autoimmune rheumatic disorders. Scand J Rheumatol 1989, 18, 69–76.
Kobayashi, S., Shimamoto, T., Taniguchi, O., Hashimoto, H., Hirose, S. Klinefelter's syndrome associated with progressive systemic sclerosis: Report of a case and review of the literature. Clin Rheumatol 1991, 10, 82–86.
Nakano, Y., Sato, A., Iwata, M., et al. Pulmonary adenocarcinoma and rheumatoid arthritis with interstitial pneumonia in a patient with Klinefelter's syndrome. Nippon Kyobusikkan Gakkai Zasshi 1991, 29, 495–499 (abstract in English).
Jungers, P., Dougados, M., Pélissier, C., et al. Influence of oral contraceptive therapy on the activity of systemic lupus erythematosus. Arthritis Rheum 1982, 25, 618–623.
Hazes, J.M.W., Van Zeben, D. Oral contraception and its possible protection against rheumatoid arthritis. Ann Rheum Dis 1991, 50, 72–74.
Schweikert, H.U., Weissbach, L., Stangenberg, C., et al. Clinical and endocrinological characterization of two subjects with Reifenstein syndrome associated with qualitative abnormalities of the androgen receptor. Horm Res 1987, 25, 72–79.
Balestrazzi, P., Ferraccili, G.F., Ambanelli, U., Giovannelli, G. Juvenile rheumatoid arthritis in Turner's syndrome. Clin Exp Rheumatol 1986, 4, 61–62.
Mazes, E., Fuchs, S. Linkage between immune response potential to DNA and X chromosome. Nature 1974, 249, 167–168.
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Kobayashi, S., Yamamoto, S., Tanaka, M. et al. Klinefelter's syndrome and rheumatoid arthritis report of a case and review of the literature. Clin Rheumatol 13, 500–503 (1994). https://doi.org/10.1007/BF02242951
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DOI: https://doi.org/10.1007/BF02242951