Mizuma Atsushi, Kouchi Maiko, Kijima Chikage, Yutani Sachiko, Uesugi Tsuyoshi, Nagata Eiichiro, Takizawa Shunya
Department of Neurology, Tokai University School of Medicine, Isehara, Japan.
Cent Eur J Immunol. 2018;43(4):490-494. doi: 10.5114/ceji.2018.81349. Epub 2018 Dec 31.
We report an 82-year-old man with recurrence of Mikulicz's disease accompanied with mononeuritis multiplex. On admission, both upper eyelids, the salivary gland, the dorsum of the left hand and both legs were swollen. Neurological examination showed motor weakness of distal limbs (manual muscle testing 3/5) and decreased touch, pain and vibration sensation of the dorsum of the left hand and both legs. Deep tendon reflex in both legs was also decreased. We diagnosed Mikulicz's disease based on high serum immunoglobulin (Ig)G4 (630 mg/dl, 26.1% of total IgG) and lacrimal gland biopsy findings. Clinical symptoms and motor conduction study findings improved after steroid therapy. However, tapering of the steroid dose resulted in recurrence two years later. Steroid therapy is usually effective for IgG4-related neuropathy, and we found that an increase of steroid dose was effective to treat the recurrence. But, in general, a suitable maintenance dose of steroid in combination with an immunosuppressant may be necessary to prevent relapse.
我们报告了一名82岁男性，患有米库利奇病复发并伴有多灶性单神经炎。入院时，双侧上眼睑、唾液腺、左手背及双下肢均肿胀。神经系统检查显示远端肢体运动无力（徒手肌力测试3/5），左手背及双下肢触觉、痛觉和振动觉减退。双下肢的腱反射也减弱。我们根据高血清免疫球蛋白（Ig）G4（630mg/dl，占总IgG的26.1%）及泪腺活检结果诊断为米库利奇病。类固醇治疗后临床症状及运动传导研究结果有所改善。然而，两年后类固醇剂量逐渐减少导致疾病复发。类固醇治疗通常对IgG4相关性神经病变有效，我们发现增加类固醇剂量对治疗复发有效。但是，一般来说，可能需要合适剂量的类固醇维持治疗并联合免疫抑制剂以预防复发。
