Severe unilateral orbitopathy in a patient with Hashimoto’s thyroiditis - a case report

Abstract

BackgroundThyroid-associated orbitopathy (TAO) constitutes an immune-mediated inflammation of the orbital tissues of unclear etiopathogenesis. TAO is most prevalent in hyperthyroid patients with Graves’ disease (GD); however, severe cases of orbitopathy associated with Hashimoto’s thyroiditis (HT) have rarely been described.Case presentationHerewith we report an unusual case of a middle-aged clinically and biochemically euthyroid woman with a stable HT, who developed a severe unilateral left-sided TAO. Thyrotropin receptor antibodies (TRAb) concentration was negative. Intraocular pressure in the left eye was mildly elevated (24 mmHg), while vision acuity was not compromised. Abnormal positioning of the eyeball suggested the extraocular muscles involvement. Unilaterally, von Graefe’s, Stellwag’s, Kocher’s and Moebius' signs were positive. Conjunctival erythema, redness and edema of the eyelid and an enlarged, swollen lacrimal caruncle were visible. She received 4/7 points in the Clinical Activity Scale (CAS) and class IV in the NO SPECS severity scale for the left eye (I-0, II-a, III-0, IV-b, V-0, VI-0). Magnetic resonance imaging (MRI) revealed thickening of the left medial rectus muscle with an increase in T2 signal intensity and prolonged T2 relaxation indicating an active form of TAO. The patient received therapy with glucocorticosteroids intravenously, followed by intramuscular injections with a cumulative dose of 3.24 g of methylprednisolone during a 9-week period with good tolerance. The applied therapy, combined with adequate L-thyroxine substitution, as well as vitamin D and selenium supplementation, resulted in a complete remission of ophthalmic symptoms.ConclusionsUnilateral exophthalmos in TRAb-negative patients with HT is not a typical manifestation of the disease, and requires a wider differential diagnosis with MRI of the orbits. Scheme of three iv. pulses of methylprednisolone intravenously and the continuation of treatment with im. injections seems to be an effective and safe method of treatment in this group of patients. What is more, adequate vitamin D supplementation and the maintenance of biochemical euthyroidism may help to achieve an ultimate therapeutic effect. Patients with TAO in the course of HT need a careful and continued interdisciplinary approach both ophthalmological and endocrinological. Further studies are needed to elucidate the etiopathogenesis of TAO in TRAb-negative patients.

Unusual onset of thyroid associated orbitopathy during pregnancy: case report and review of literature

“…The usual rapid volume redistribution after delivery may explain the immediate improvement of TAO. It is not entirely clear why some of the TAO patients develop asymmetric disease; at presentation, other causes of unilateral exophthalmos have to be ruled out [34]. We [35] and others found that the orbital process is unequivocally two-sided by imaging, even in those cases where one side appears completely spared.…”

IgG4 as a Biomarker in Graves’ Orbitopathy

“…Many conditions may mimic GO, including orbital pseudotumor; orbital neoplasms, both malignant (e.g., lymphomas) and benign; infections; orbital myositis; and inflammatory orbitopathy, e.g., granulomatosis with polyangiitis or sarcoidosis [56]. Orbitopathy can also occur in other autoimmune thyroid diseases like Hashimoto's thyroiditis, but it is exceptionally rare compared to the incidence rate of GD [57]. Therefore, the differential diagnosis in patients with eye proptosis or periorbital edema should be performed in each case.…”

Thyroid eye disease: Redefining its management—A review

“…10,11 2 | EPIDEMIOLOGY TED is associated with Grave's disease hyperthyroidism in the majority (90%) of cases, but can also be found in euthyroidism, Hashimoto's disease and primary hypothyroidism. 2,[12][13][14] Systemic Grave's disease affects approximately 1% of the adult population and prevalence of TED among Grave's patients is approximately 20% to 50%. 15,16 There is no distinct ethnic predisposition.…”