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Prof. Dr. med. Christoph Hintschich, FEBO
University Eye Hospital, Ludwig-Maximilian University Munich
Mathildenstrasse 8
DE–80336 Munich (Germany)
E-Mail [email protected]
Wiersinga WM, Kahaly GJ (eds): Graves’ Orbitopathy: A Multidisciplinary Approach – Questions and Answers.
Basel, Karger, 2017, pp 26–32 (DOI: 10.1159/000475945)
___________________
Michele Marinò
Endocrinology Unit I, Department of Clinical and Experimental Medicine, University of Pisa and University Hospital of Pisa, Pisa, Italy
Should This Condition Always Be Called Graves’ Orbitopathy?
The eye disease generally associated with Graves’ disease has been known by many names [1]. The various definitions of the disease are reported in Table 1. The various names reflect: (i) the presence and variability of the thyroid dysfunction associated with the eye disease, as discussed in detail below; (ii) the fact that the pathogenesis of the condition is not fully elucidated; (iii) the fact that the disease has many and varied clinical presentations. We believe that the term Graves’ orbitopathy (GO) is an accurate reflection of the condition in the majority of patients. It does usually occur in the context of Graves’ disease. It does involve parts of the orbit so that it is reasonable to use the term orbitopathy. However, it could be argued that in the mild form of the condition, where the symptoms and signs are predominantly related to periorbital soft tissues, the term orbitopathy may be an alarming misnomer. From a practical point of view, in the Graves’ eye clinic the term GO is appropriate.
Is Graves’ Orbitopathy Exclusively Occurring in Patients with Graves’ Hyperthyroidism?
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
|