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Klinefelter’s syndrome associated with systemic lupus erythematosus and autoimmune hepatitis

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Modern Rheumatology

Abstract

Klinefelter’s syndrome (KFS) tends to be associated with immunological disorders. We describe a 37-year-old man who presented signs of testicular atrophy and decreased body hair. He showed pancytopenia and elevated levels of liver enzymes. Chromosome analysis revealed 47XXY karyotype; therefore, he was diagnosed with KFS, with systemic lupus erythematosus and autoimmune hepatitis. Treatment with a high dose of methylprednisolone and methyltestosterone improved thrombocytopenia and symptoms, suggesting that methyltestosterone may have a clinical benefit in the treatment of KFS with a low level of testosterone accompanying immunological disorders.

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Correspondence to Kohei Yamauchi.

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Sasaki, N., Yamauchi, K., Sato, R. et al. Klinefelter’s syndrome associated with systemic lupus erythematosus and autoimmune hepatitis. Mod Rheumatol 16, 305–308 (2006). https://doi.org/10.1007/s10165-006-0511-5

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