Other polyendocrine autoimmunity syndromes

Rarely, diabetes mellitus results from a decreased biological response to a normal amount of insulin. In type A insulin resistance, the problem lies in the insulin receptor, which is quantitatively or qualitatively abnormal, but in the type B syndrome autoantibodies to the insulin receptor produce diabetes mellitus. In vitro, using short-term culture experiments, these antibodies mimic the action of insulin, which may account for the occurrence of hypoglycemia in some patients. More typically, however, there is massive insulin resistance, so that even 15 000 units of insulin per day may not lower blood glucose, and prolonged in vitro experiments reveal the antagonistic properties of the antibodies. About one-third of these patients have other autoimmune diseases, including systemic lupus erythematosus, Sjogren's syndrome, scleroderma, primary biliary cirrhosis,

Hashimoto's thyroiditis and autoimmune thrombocytopenia. Serological markers include a raised erythrocyte sedimentation rate (HSR), hypergammaglobulinemia and hypocomplementemia.

The POEMS syndrome is a rare form of plasma cell dyscrasia with polyneuropathy, organomegaly (liver and/or spleen), endocrinopathy (hypogonadism, hypothyroidism or diabetes mellitus), monoclonal gammopathy and skin changes, these features giving rise to the acronym. It is likely that the POEMS syndrome represents a variant of osteosclerotic myeloma. The endocrine dysfunction may be caused by specific autoantibodies but this remains unproven.

See also: Adrenal autoimmunity; Autoimmune diseases; Candida, infection and immunity; insulin-dependent diabetes mellitus, human; Systemic lupus erythematosus (SLE), human; Thyroid autoimmunity, human; Vitiligo.

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