Klinefelter's syndrome associated with autoimmune disease

Abstract

The striking preponderance of systemic lupus erythematosus (SLE) in females is a constant reminder of the importance of the sex-hormonal factor in the multifactorial pathogenesis of autoimmunity. This is supported by several experimental studies in lupus-prone mice and in vitro demonstrating a faciltiating effect of oestrogens on the immune system, predisposing to autoimmunity, while androgens may have a protective effect. In this light, the association of SLE and Klinefelter's syndrome is of special interest. We report another example of the association in which the findings of leukopenia in an elderly patient led to the diagnosis of both conditions.