Kamiya Mari, Shane Peter Y, Soejima Makoto, Tohda Shuji, Miyasaka Nobuyuki, Kohsaka Hitoshi
Department of Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), Japan.
Intern Med. 2016;55(10):1355-61. doi: 10.2169/internalmedicine.55.5332. Epub 2016 May 15.
A 68-year-old man was admitted because of weakness of the left leg, dysesthesiae of the extremities and bilateral lower extremity purpura. A neurological examination showed mononeuritis multiplex with laboratory evidence of hypocomplementemia, cryoglobulinemia and leukocytoclastic vasculitis in the biopsy of a skin specimen. The patient also exhibited bilateral submandibular gland swelling, elevated serum IgG4 levels and infiltration of a large number of IgG4-positive plasma cells in the submandibular glands. These findings were consistent with both cryoglobulinemic vasculitis and IgG4-related disease. The administration of oral prednisolone (1 mg/kg/day) resolved the neurological manifestations and the swelling of the submandibular glands and cryoglobulinemia.
一名68岁男性因左腿无力、肢体感觉异常和双侧下肢紫癜入院。神经系统检查显示多发性单神经炎，皮肤活检的实验室证据显示有低补体血症、冷球蛋白血症和白细胞破碎性血管炎。患者还表现出双侧下颌下腺肿大、血清IgG4水平升高以及下颌下腺大量IgG4阳性浆细胞浸润。这些发现与冷球蛋白血症性血管炎和IgG4相关疾病均相符。口服泼尼松龙（1毫克/千克/天）治疗后，神经症状、下颌下腺肿胀及冷球蛋白血症均得到缓解。
