The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
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A Case of Rheumatoid Arthritis Associated with Klinefelter's Syndrome

클라인펠터 증후군(Klinefelter's Syndrome)에서 동반된 류마티스관절염 1예

  • Seo, Mi-Ryoung (Department of Internal Medicine, Division of Rheumatology, Gil Medical Center, Gachon University of Medicine and Science) ;
  • Cho, Hyun-Jung (Department of Internal Medicine, Division of Rheumatology, Gil Medical Center, Gachon University of Medicine and Science) ;
  • Choi, Hyo-Jin (Department of Internal Medicine, Division of Rheumatology, Gil Medical Center, Gachon University of Medicine and Science) ;
  • Baek, Han-Joo (Department of Internal Medicine, Division of Rheumatology, Gil Medical Center, Gachon University of Medicine and Science) ;
  • Ahn, Jeong-Yeal (Department of Laboratory Medicine, Gil Medical Center, Gachon University of Medicine and Science)
  • 서미령 (가천의과대학교 길병원 내과학교실 류마티스내과) ;
  • 조현정 (가천의과대학교 길병원 내과학교실 류마티스내과) ;
  • 최효진 (가천의과대학교 길병원 내과학교실 류마티스내과) ;
  • 백한주 (가천의과대학교 길병원 내과학교실 류마티스내과) ;
  • 안정열 (가천의과대학교 길병원 진단검사의과학교실)
  • Published : 2011.09.01
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Abstract

Klinefelter's syndrome is a disorder of sexual differentiation in males, characterized by the presence of two or more X-chromosomes, hypogonadism, and lack of secondary sexual characteristics. The association between Klinefelter's syndrome and systemic lupus erythematous has been described, while cases of rheumatoid arthritis associated with Klinefelter's syndrome are rare. We report the first Korean case: a 29-year-old man with Klinefelter's syndrome who developed rheumatoid arthritis. The sex hormone imbalance might have influenced the onset and course of his disease.

저자 등은 29세 남자에서 드문 활동성 류마티스관절염이 클라인펠터 증후군에 동반하여 발생한 국내 첫 증례를 경험하였다. 환자는 클라인펠터 증후군의 치료를 위해 지속적인 테스토스테론의 보충 요법을 했지만, 혈청 안드로겐 수치는 정상보다 낮았다. 환자의 성호르몬 불균형 상태가 류마티스 관절염의 발병과 경과에 영향을 미쳤을 것으로 추정된다.

Keywords

References

  1. Smyth CM, Bremner WJ. Klinefelter syndrome. Arch Intern Med 1998;158:1309-1314. https://doi.org/10.1001/archinte.158.12.1309
  2. Rovensky J, Imrich R, Lazurova I, Payer J. Rheumatic diseases and Klinefelter's syndrome. Ann N Y Acad Sci 2010;1193:1-9. https://doi.org/10.1111/j.1749-6632.2009.05292.x
  3. Macsween RN. Reticulum-cell sarcoma and rheumatoid arthritis in a patient with XY/XXY/XXXY Klinefelter's syndrome and normal intelligence. Lancet 1965;27:460-461.
  4. Kobayashi S, Yamamoto S, Tanaka M, Hashimoto H, Hirose S. Klinefelter's syndrome and rheumatoid arthritis: report of a case and review of the literature. Clin Rheumatol 1994;13:500-503. https://doi.org/10.1007/BF02242951
  5. Al-Arfaj HF. Klinefelter's syndrome and rheumatoid arthritis: report of a case and review of the literature. Int J Rheum Dis 2010;13:86-88. https://doi.org/10.1111/j.1756-185X.2009.01452.x
  6. Lee YJ, Choe YJ, Shin KC, Lee EB, Kim HA, Song YW. A case of Klinefelter's syndrome associated with systemic lupus erythematosus. Korean J Med 2000;59:331-334.
  7. Lipsky PE. Rheumatoid arthritis. In: Fauci AS, Braunwald E, Kasper DL, Hauser SL, Longo DL, Jameson JL, Loscalzo J, eds. Harrison's Principles of Internal Medicine. 17th ed. New York: McGraw-Hill, 2008:2083-2092.
  8. Symmons DP, Barrett EM, Bankhead CR, Scott DG, Silman AJ. The incidence of rheumatoid arthritis in the United Kingdom: results from the Norfolk Arthritis Register. Br J Rheumatol 1994;33:735-739. https://doi.org/10.1093/rheumatology/33.8.735
  9. Arnett FC, Edworthy SM, Bloch DA, et al. The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum 1988;31:315-324. https://doi.org/10.1002/art.1780310302
  10. Waldburger JM, Firestein GS. Rheumatoid arthritis. In: Klippel JH, Stone JH, Crofford LJ, White PH, eds. Primer on the Rheumatic Diseases. 13th ed. New York: Springer, 2008:122-124.
  11. Iikuni N, Sato E, Hoshi M,et al. The influence of sex on patients with rheumatoid arthritis in a large observational cohort. J Rheumatol 2009;36:508-511. https://doi.org/10.3899/jrheum.080724
  12. Ostensen M, Villiger PM. The remission of rheumatoid arthritis during pregnancy. Semin Immunopathol 2007;29:185-191. https://doi.org/10.1007/s00281-007-0072-5
  13. Goemaere S, Ackerman C, Goethals K, et al. Onset of symptoms of rheumatoid arthritis in relation to age, sex and menopausal transition. J Rheumatol 1990;17:1620-1622.
  14. Tengstrand B, Carlstrom K, Fellander-Tsai L, Hafstrom I. Abnormal levels of serum dehydroepiandrosterone, estrone, estradiol in men with rheumatoid arthritis: high correlation between serum estradiol and current degree of inflammation. J Rheumatol 2003;30:2338-2343.
  15. Tengstrand B, Carlstrom K, Hafstrom I. Gonadal hormones in men with rheumatoid arthritis: from onset through 2 years. J Rheumatol 2009;36:887-892. https://doi.org/10.3899/jrheum.080558
  16. de la Torre B, Hedman M, Nilsson E, Olesen O, Thorner A. Relationship between blood and joint tissue DHEAS levels in rheumatoid arthritis and osteoarthritis. Clin Exp Rheumatol 1993;11:597-601.
  17. Cutolo M, Seriolo B, Villaggio B, Pizzorni C, Craviotto C, Sulli A. Androgens and estrogens modulate the immune and inflammatory responses in rheumatoid arthritis. Ann N Y Acad Sci 2002;966:131-142. https://doi.org/10.1111/j.1749-6632.2002.tb04210.x
  18. Bizzarro A, Valentini G, Di Marino G, Daponte A, De Bellis A, Iacono G. Influence of testosterone therapy on clinical and immunological features of autoimmune disease associated with Klinefelter's syndrome. J Clin Endocrinol Metab 1987;64:32-36. https://doi.org/10.1210/jcem-64-1-32
  19. Cutolo M, Balleari E, Giusti M, Intra E, Accardo S. Androgen replacement therapy in male patients with rheumatoid arthritis. Arthritis Rheum 1991;34:1-5. https://doi.org/10.1002/art.1780340102