Department of Ophthalmology, Asahikawa Medical University, Asahikawa, Hokkaido, Japan.
Medicine and Engineering Combined Research Institute, Asahikawa Medical University, Midorigaoka Higashi 2-1-1-1, Asahikawa, Hokkaido, 078-8510, Japan.
J Med Case Rep. 2021 Dec 29;15(1):618. doi: 10.1186/s13256-021-03207-4.
Dysthyroid optic neuropathy is the most commonly suspected diagnosis of optic neuropathy in Graves' patients; however, other causes need to be ruled out. We present a unique case of optic neuropathy secondary to hypertrophic pachymeningitis with antineutrophil cytoplasmic antibody-associated vasculitis, which was suspected to be antithyroid drug related.
A 79-year-old Japanese male presented with acute visual loss in the left eye. He had a 24-year history of Graves' disease and was taking methimazole. Best-corrected visual acuity was 0.8 in the right eye and light perception in the left eye, and relative afferent pupillary defect in the left eye was seen. Ocular movement was normal, and there were no findings explaining visual loss in intermediate optic media and fundus in the left eye. Contrast-enhanced magnetic resonance imaging demonstrated thickened dura mater. Tests for myeloperoxidase-antineutrophil cytoplasmic antibody, proteinuria, and hematuria were positive; pulmonary nodule lesions and a blood clot in the left lower leg were also found. After excluding the presence of diseases that could lead to hypertrophic pachymeningitis, we diagnosed optic neuropathy due to hypertrophic pachymeningitis with granulomatosis with polyangiitis-a subtype of antineutrophil cytoplasmic antibody-associated vasculitis. Since he had history of using methimazole, antineutrophil cytoplasmic antibody-associated vasculitis was considered as drug related. We started high-dosage steroid pulse therapy followed by 1 mg/kg body weight daily of oral prednisolone, and subsequently tapered. Methimazole was stopped. Best-corrected visual acuity recovered to 0.9, 2 weeks after starting treatment. Though myeloperoxidase-antineutrophil cytoplasmic antibody remained negative, the symptom relapsed 6 months after treatment initiation. We gave a second high-dose steroid pulse therapy followed by prednisolone tapered together with methotrexate. Remission remained, and using 4 mg/week methotrexate without prednisolone, myeloperoxidase-antineutrophil cytoplasmic antibody was kept within the normal limit until now, 4 years after onset.
We present a case of optic neuropathy with hypertrophic pachymeningitis related to antineutrophil cytoplasmic antibody-associated vasculitis, which was suspected to be drug related. The patient had good visual recovery after quitting the drug and receiving immunosuppressive therapy with systemic steroids. Hypertrophic pachymeningitis with antineutrophil cytoplasmic antibody-associated vasculitis related to antithyroid drugs should be considered as a differential diagnosis for optic neuropathy in Graves' patients in whom optic nerve compression is not obvious.
在格雷夫斯病患者中，最常怀疑的视神经病变诊断是甲状腺相关眼病；然而，需要排除其他原因。我们报告了一例继发于伴有抗中性粒细胞胞质抗体血管炎的肥厚性硬脑膜炎的视神经病变，该病变被怀疑与抗甲状腺药物有关。
一名 79 岁的日本男性左眼急性视力丧失。他患有格雷夫斯病 24 年，正在服用甲巯咪唑。右眼最佳矫正视力为 0.8，左眼光感，左眼相对传入瞳孔缺陷。眼球运动正常，左眼中间视神经和眼底未见解释视力丧失的发现。对比增强磁共振成像显示硬脑膜增厚。髓过氧化物酶-抗中性粒细胞胞质抗体、蛋白尿和血尿检测阳性；还发现肺部结节病变和左小腿血块。排除可能导致肥厚性硬脑膜炎的疾病后，我们诊断为伴有肉芽肿性多血管炎的肥厚性硬脑膜炎-抗中性粒细胞胞质抗体相关血管炎的视神经病变。由于他有使用甲巯咪唑的病史，因此考虑抗中性粒细胞胞质抗体相关血管炎与药物有关。我们开始大剂量类固醇脉冲治疗，随后每天口服 1mg/kg 体重的泼尼松龙，并逐渐减量。停用甲巯咪唑。开始治疗后 2 周，最佳矫正视力恢复至 0.9。虽然髓过氧化物酶-抗中性粒细胞胞质抗体仍为阴性，但治疗开始后 6 个月症状复发。我们给予第二次大剂量类固醇脉冲治疗，随后给予泼尼松龙减量联合甲氨蝶呤。缓解仍在继续，在没有泼尼松龙的情况下，每周使用 4mg 甲氨蝶呤，到现在，发病 4 年后，髓过氧化物酶-抗中性粒细胞胞质抗体仍在正常范围内。
我们报告了一例与抗中性粒细胞胞质抗体相关血管炎相关的伴有抗中性粒细胞胞质抗体相关血管炎的视神经病变，该病变被怀疑与药物有关。停药和接受全身类固醇免疫抑制治疗后，患者视力恢复良好。对于视神经压迫不明显的格雷夫斯病患者，如果怀疑为视神经病变，应考虑甲状腺相关药物引起的肥厚性硬脑膜炎伴抗中性粒细胞胞质抗体相关血管炎作为鉴别诊断。
