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Although GO is usually associated with overt Graves’ hyperthyroidism, a minority of patients do not have hyperthyroidism [2, 3]. In approximately 40% of such cases, this simply reflects the occurrence of GO before the onset of hyperthyroidism (Fig. 1). Thus, some patients develop overt hyperthyroidism within a period of 18–30 months after the appearance of GO [2]. Nevertheless, in about 5% of patients with GO, hyperthyroidism is not observed at all [2, 3]. This subgroup includes (i) patients with hypothyroidism due to autoimmune thyroiditis and (ii) patients with a normal thyroid function but with biochemical evidence of thyroid autoimmunity [detectable circulating thyroid autoantibodies, especially against the TSH receptor (TSH-R)] or subclinical evidence of hyperthyroidism (a reduced TSH or a TSH not adequately responsive to TRH). The latter condition is usually referred to as “euthyroid GO.” In a study conducted in a tertiary referral centre in a large series of consecutive GO patients, about 3% had hypothyroid autoimmune thyroiditis, and approximately 2% had euthyroid GO [4]. In the same study the severity of GO did not seem to vary based on the underlying thyroid condition, although it was previously observed that GO may be more severe in patients with hypothyroidism [5]. The diagnosis of euthyroid GO must be made quite carefully, as other conditions, including orbital lymphoma, may resemble GO.

Fig. 1. Temporal relationship between the onset of hyperthyroidism and the onset of Graves’ orbitopathy (GO). Modified from Means [1].

Table 1. Synonyms for Graves’ orbitopathy

Graves’ eye disease

Graves’ ophthalmopathy

Ophthalmic Graves’ disease

Thyroid-associated ophthalmopathy

Thyroid exophthalmos

Thyroid eye disease

Thyroid-related eye disease

Von Basedow ophthalmopathy