ABSTRACT

Graves’ disease is a multisystem disorder, which can manifest itself as Graves’ hyperthyroidism, Graves’ ophthalmopathy (GO), localized myxedema, and thyroid acropachy. Graves’ hyperthyroidism is the most prevalent phenotype with a female to male ratio of 8:1. Graves’ ophthalmopathy is less prevalent with a sex ratio of 5.5:1, occurring mostly in association with Graves’ hyperthyroidism but hyperthyroidism is absent in about 20% of patients presenting with GO. Localized myxedema is rare with a sex ratio of 3.5:1, almost always occurring together with Graves’ hyperthyroidism

and to a lesser extent with GO. Thyroid acropachy is a very rare condition with an equal sex distribution of 1:1, seen almost exclusively in patients who also express the other phenotypes of Graves’ disease. When present, GO usually manifests itself in the first year after the onset of Graves’ hyperthyroidism, localized myxedema in the second year and thyroid acropachy in the third year. Serum concentrations of thyroid stimulating immunoglobulins (TSI), the hallmark of Graves’ disease, are generally higher in Graves’ hyperthyroid patients with GO than in Graves’ hyperthyroid patients without GO, and are even higher in patients who also have the other two phenotypes. The question arises whether the differential expression of phenotypes in Graves’ disease patients just reflects the severity of the autoimmune attack, or that the phenotypes constitute different albeit closely related disease entities.