Frazzetto Giuseppe, Arpi Maria Luisa, Regalbuto Concetto, Belfiore Antonino, Frasca Francesco, Le Moli Rosario
University of Catania, Department of Clinical and Experimental Medicine, Endocrinology Unit, Garibaldi-Nesima Hospital, Catania, 95122, Italy.
Int Med Case Rep J. 2023 Apr 1;16:233-237. doi: 10.2147/IMCRJ.S397007. eCollection 2023.
Graves' ophthalmopathy (GO) is an autoimmune manifestation of orbit affecting approximately 25% of patients with Graves' disease (GD). Autoreactive T cells involved in thyroid autoimmunity can recognize the thyroid-stimulating receptor (TSHr) expressed in orbital tissues of GO patients. Clinical manifestations of GO are rather different depending on the presence of some risk factors, such as smoking, hyperthyroidism duration, age, biological activity of anti-TSHr antibodies (TSH-R-Ab) and metabolic diseases.
Here, we present a rare case of euthyroid single muscular GO in a 50-year-old patient who was a smoker and had dyslipidemia for several years. The patient experienced a very rapid and severe depression of ocular motility of the right eye that caused uncorrectable and constant diplopia, severely affecting his quality of life. He was euthyroid, and TSH-R-Ab plasmatic levels were only slightly elevated. Intravenous corticosteroid pulse therapy was partially effective, and two rounds of wall orbital surgical decompression were necessary. Massive mono-muscular fibrosis was evidenced by biopsy of the right inferior rectus muscle.
Severe unilateral, mono-muscular GO in a euthyroid Graves' patient was found to be sustained by rapid and massive fibrosis of the inferior rectus muscle of the right orbit. Clarification of the pathogenetic mechanisms of these GO clinical forms requires further studies.
格雷夫斯眼病(GO)是一种眼眶自身免疫性表现,约25%的格雷夫斯病(GD)患者会出现。参与甲状腺自身免疫的自身反应性T细胞可识别GO患者眼眶组织中表达的促甲状腺素受体(TSHr)。GO的临床表现因一些危险因素的存在而有很大差异,如吸烟、甲亢病程、年龄、抗TSHr抗体(TSH-R-Ab)的生物学活性和代谢疾病。
在此,我们报告一例罕见的50岁甲状腺功能正常的单肌型GO病例,该患者有吸烟史且患有血脂异常数年。患者右眼眼球运动迅速且严重受限,导致不可矫正的持续性复视,严重影响其生活质量。他甲状腺功能正常,TSH-R-Ab血浆水平仅略有升高。静脉注射糖皮质激素脉冲治疗部分有效,需要进行两轮眼眶壁手术减压。右眼下直肌活检证实有大量单肌型纤维化。
甲状腺功能正常的格雷夫斯病患者出现严重的单侧单肌型GO,发现是由右眼眶下直肌迅速大量纤维化所致。阐明这些GO临床类型的发病机制需要进一步研究。