Endocrine Abstracts

Introduction: Graves’ ophthalmopathy (GO) is common in the course of Graves’ disease, but optic neuropathy is present in only ~5% of patients with GO. We present a case of recalcitrant Graves’ orbitopathy with late development of neuropathy, satisfactorily treated with rituximab.

Case report: A 50-year-old female smoker was referred to our department for further management of Graves’ disease with associated GO. She complained of worsening eyelid edema, intermittent vertical diplopia, and pruritic eyes for several months prior, but diagnosis remained elusive due to the predominance of eyelid swelling and inconspicuous hyperthyroid symptoms.

On presentation she had disfiguring eyelid edema, bilateral exophthalmos (24/110/24), mild diplopia, normal visual acuity, color perception, and fundoscopy. CAS score was 5/7, with moderate severity. She received i.v. pulse methylprednisolone for 3 months to a cumulative dose of 6 g with improvement in CAS to 3/7. Standard orbital radiotherapy was delivered 3 months later, with minor improvement of disease activity.

Thyroid status stabilized on a combination of methimazole plus adjunct lithium, permitting an uncomplicated thyroidectomy 2 months later.

However, visual acuity declined in the course of the above treatments to ‘hand motion’, necessitating ‘salvage’ rituximab infusions twice, at 375 mg/m². CD20 depletion was immediate and sustained after 6 months, but thyroid-stimulating immunoglobulin titers remained high throughout. 6 months after treatment CAS is 1/7, color vision is improved and visual acuity measures 4/10 bilaterally.

Conclusions: i) GO may not be readily recognized if any particular feature prevails in the clinical picture.

ii) Optic neuropathy can develop after GO treatment, despite improved disease activity and in the presence of normal disc appearance.

iii) Late administration of rituximab after established anti-inflammatory therapy may still benefit vision.