Endocrine Abstracts

Background: Graves’ orbitopathy (GO) is the major extrathyroidal manifestation of Graves’ disease (GD). Even if usually mild and non-progressive, in its worst forms, GO can represent a major therapeutic challenge, because is not always responsive to the available medical treatments. First line treatment for moderate-to-severe and active GO relies on intravenous (i.v.) methylprednisolone. Optic neuropathy is a sight-threatening complication which can seriously impact the quality of life and should be immediately treated. Here we present a complicated case of worsening and aggressive GO.

Case report: In March 2022 a 78-years-old woman presented to the endocrinological outpatient clinic for newly diagnosed hyperthyroidism. Positive TSH receptor antibodies (TrAb) confirmed GD diagnosis. In medical history: atrial fibrillation on amiodarone therapy (discontinued in June 2021 because of hypothyroidism) and high blood pressure. The third dose of anti-COVID-19 vaccine was received in December 2021. During the first visit, mild ocular involvement was detected, therefore oral prednisone (25 mg per day) was prescribed together with methimazole (20 mg per day) and selenium supplementation. After 40 days, a rapid worsening of the ocular pattern occurred. The eye examination showed bilateral exophthalmos, eyelid oedema, conjunctival chemosis, with a reduced ocular motility and visual acuity. Therefore, i.v. methylprednisolone was planned, with a cumulative dose of 4.5 g in 12 weekly infusions (6 of 0.5 g plus 6 of 0.25 g). Nonetheless, after the second infusion, the orbit MRI showed diffuse hypertrophy of the oculomotor muscles with incarceration of the optic nerves. Considering the severity and evolutionary nature of the GO, in June 2022 the patient underwent urgent orbital decompression surgery, and daily 0.5 g methylprednisolone i.v. was prescribed in the perioperative days. After discharge, treatment with i.v. methylprednisolone was continued and cyclosporine (100 mg daily) was added to the treatment, with a significant clinical improvement, except for visual acuity. From August 2022, scalar therapy with oral prednisone together with ciclosporine was planned up to the maintenance dosage. The latest eye re-evaluation found in right eye only light perception and in left eye a perception of 2/30. Orthoptic rehabilitation was finally planned, although very little chance of recovery.

Conclusions: Although rare, GO can occur in acute and rapidly progressive forms, even in patients with mild hyperthyroidism and low TrAb levels at diagnosis. Since a sudden worsening can arise, GO patients should be carefully and strictly observed, hopefully in a centre equipped for multidisciplinary (both clinical and surgical) management.