J Korean Endocr Soc.  2008 Oct;23(5):352-357. 10.3803/jkes.2008.23.5.352.

Primary Hypogonadism Associated with Ankylosing Spondylitis

• Jun BY
• Lee GJ
• Kim JH
• Lee JM
• Chang SA

• KMID: 1479230
• DOI: http://doi.org/10.3803/jkes.2008.23.5.352

Abstract

Male patients with hypogonadism have an increased risk of developing rheumatic diseases. Most causes of hypogonadism related with rheumatic disease are karyotype abnormality such as Klinefelter's syndrome or Turner's syndrome and gonadal dysgenesis. A 24-year-old year male was admitted for pain of both hip joints that had worsened over 2 months. He had hip joint involvement from ankylosing spondylitis and did not show secondary sex characteristics. His sex hormones and gonadotropins levels indicated hypergonadotropic hypogonadism. The karyotype was 46 XY, and there was no obvious cause of hypogonadism. Here we report on clinical features of this first Korean case of primary hypogonadism accompanying ankylosing spondylitis.

Keyword

ankylosing spondylitis; hypergonadotropic hypogonadism; rheumatic disease

MeSH Terms

Gonadal Dysgenesis
Gonadal Steroid Hormones
Gonadotropins
Hip Joint
Humans
Hypogonadism
Karyotype
Klinefelter Syndrome
Male
Rheumatic Diseases
Sex Characteristics
Spondylitis, Ankylosing
Turner Syndrome
Young Adult
Gonadal Steroid Hormones
Gonadotropins

Figure

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  Fig. 1 Clinical feature of the patient. He showed obese build and gynecomastia, axillary hair loss, genu valgum and he had a small penis and scrotums.

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  Fig. 2 X-ray test of hip joint. Marginal erosive change with sclerosis at acetabular side of both hip joint is noted with mild space narrowing.

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  Fig. 3 T2-weighted magnetic resonance images through the sacroiliac joints. Arrows showed enhancement at the both sacroiliac joints, indicating active sacroiliitis.

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  Fig. 4 Sonography of testes. Too small testes are noted (Lt. 1.3 × 0.5 cm, Rt. 1.5 × 0.5 cm) and subcutaneous fat is very thick.

Reference

1. Styne D. Gardner DG, Shoback D, editors. Puberty. Greenspan's Basic & Clinical Endocrinology. 2007. 8th ed. New York: Mcgraw-Hill Co;620–629.

2. Jimenéz-Balderas FJ, Tápia-Serrano R, Fonseca ME, Arellano J, Beltrán A, Yáñez P, Camargo-Coronel A, Fraga A. High frequency of association of rheumatic/autoimmune diseases and untreated male hypogonadism with severe testicular dysfunction. Arthritis Res. 2001. 3:362–367.

3. Vallotton MB, Forbes AP. Autoimmunity in gonadal dysgenesis and Klinefelter's syndrome. Lancet. 1967. 1:648–651.

4. Stern R, Fishman J, Brusman H, Kunkel HG. Systemic lupus erythematosus associated with Klinefelter's syndrome. Arthritis Rheum. 1977. 20:18–22.

5. Kobayashi S, Shimamoto T, Taniguchi O, Hashimoto H, Hirose S. Klinefelter's sydnrome associated with progressive systemic sclerosis: Report of a case and review of the literature. Clin Rheumatol. 1991. 10:84–86.

6. 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. l. 1994. 13:500–503.

7. Pages M, Laroche S, Lassoued S, Pages P, Mazieres B, Arlet J. The association of B27 positive spondylarthritis with Klinefelter's syndrome. Presse Med. 1990. 19:178.

8. Murakami M, Kishino B, Fushimi H, Sakata Y, Matsuyuki Y. The first report of Klinefelter's syndrome associated with polymyositis. Nippon Naika Gakkai Zasshi. 1988. 77:530–535.

9. Williams ED, Engel E, Forbes AP. Thyroiditis and gonadal dysgenesis. N Engl J Med. 1964. 270:805–810.

10. Balestrazzi P, Ferraccioli GF, Ambanelli U, Giovannelli G. Juvenile rheumatoid arthritis in Turner's syndrome. Clin Exp Rheumatol. 1986. 4:61–62.

11. Lee JH, Kim KR, Park HY, Ju JY, Chae YD, Yoon SJ, Kim KJ, Park WI, Cha BS, Song YD, Lim SK, Lee HC, Huh KB. A case of panhypopituitarism due to craniopharyngioma with slipped capitalis femoral epiphysis. J Kor Soc Endocrinol. 2002. 17:104–109.

12. Kim JD, Lee JC, Park JH. A case report of slipped capital femoral epiphysis associated with hypothyroidism and hypogonadism. J Kosin Med Coll. 1991. 7:87–94.

13. Taurog JD. Fauci AS, Braunwald E, Kasper DL, Hauser SL, Longo DL, Jameson JL, Loscalzo J, editors. The spondyloarthritides. Harrison's principles of internal medicine. 2008. 17th ed. New York: Mcgraw-Hill Co;2109–2113.

14. Bhasin S, Jameson JL. Fauci AS, Braunwald E, Kasper DL, Hauser SL, Longo DL, Jameson JL, Loscalzo J, editors. Disorders of the testis and male reproductive system. Harrison's principles of internal medicine. 2008. 17th ed. New York: Mcgraw-Hill Co;2314–2324.

15. Hammoud AO, Gibson M, Peterson CM, Hamilton BD, Carrell DT. Obesity and male reproductive potential. J Androl. 2006. 27:619–626.

16. Braun J, Sieper J. Ankylosing spondylitis. Lancet. 2007. 369:1379–1390.

17. Arniaud D, Mattei JP, Boyer J, Roux H. Sex hormones in spondylarthropathies. A study in 57 patients. Rev Rheum Engl Ed. 1998. 65:21–26.

18. Giltay EJ, Popp-Snijders C, van Schaardenburg D, Dekker-Saeys BJ, Gooren LJ, Dijkmans BA. Serum testosterone levels are not elevated in patients with ankylosing spondylitis. J Rheumatol. 1998. 25:2389–2394.

19. Gooren LJ, Giltay EJ, van Schaardenburg D, Dijkmans BA. Gonadal and adrenal sex steroids in ankylosing spondylitis. Rheum Dis Clin North Am. 2000. 26:969–987.

20. Onose G, Peretianu D, Zaharescu J, Motoiu S. Correlations between spondylarthropathic inflammatory troubles and gonadal (androgenic) troubles in men. Study on 30 cases with a new methodological analysis. Rom J Intern Med. 1995. 33:93–111.

21. Kocar IH, Yesilova Z, Ozata M, Turan M, Sengul A, Ozdemir I. The effect of testosterone replacement treatment on immunological features of patients with Klinefelter's syndrome. Clin Exp Immunol. 2000. 121:448–452.

22. Bizzarro A, Valentini G, Di Martino G, DaPonte A, De Bellis A, Iacono G. Influence of testosterone therapy on clinical and immunological features of autoimmune diseases associated with Klinefelter's syndrome. J Clin Endocrinol Metab. 1987. 64:32–36.