Aoki Asuka, Sato Keitaro, Itabashi Mitsuyo, Takei Takashi, Yoshida Takumi, Arai Junko, Uchida Keiko, Tsuchiya Ken, Nitta Kosaku
Department of Medicine, Kidney Center, Tokyo Women's Medical University, 8-1 Kawadacho, Shinjuku-ku, Tokyo, 162-8666, Japan.
Clin Exp Nephrol. 2009 Aug;13(4):367-372. doi: 10.1007/s10157-008-0127-5. Epub 2009 Jan 14.
A 48-year-old woman who had bilateral swelling in the eyelids and submandibular region was admitted. Clinical findings suggested that her renal function had deteriorated. Laboratory data showed renal insufficiency (2.52 mg/dl), hypergammaglobulinemia (IgG 3,729 mg/dl, IgA 124 mg/dl, IgM 73 mg/dl). Gallium-67 scintigram indicated abnormal uptake in bilateral lacrimal glands, submandibular glands, and kidneys. A diagnosis of Mikulicz's disease and interstitial nephritis was made, since biopsy specimens of her lacrimal gland and minor salivary gland showed diffuse infiltration of lymphocytes. In addition, renal biopsy specimens showed diffuse severe interstitial infiltration of IgG4-positive mononuclear cells. Symptoms and laboratory data normalized in response to methylprednisolone semi-pulse therapy and prednisolone 50 mg/day. Mikulicz's disease was recently reported to be IgG4 associated disease. In our case, Mikulicz's disease complicated with diffuse severe interstitial nephritis was successfully treated by corticosteroid. The present case supports the hypothesis that IgG4-related autoimmune disease could be causes of Mikulicz's disease and interstitial nephritis.
一名48岁女性因双侧眼睑和下颌下区域肿胀入院。临床检查发现其肾功能恶化。实验室数据显示肾功能不全（2.52mg/dl）、高球蛋白血症（IgG 3729mg/dl、IgA 124mg/dl、IgM 73mg/dl）。镓-67闪烁扫描显示双侧泪腺、下颌下腺和肾脏摄取异常。由于泪腺和小唾液腺活检标本显示淋巴细胞弥漫性浸润，诊断为米库利奇病和间质性肾炎。此外，肾活检标本显示IgG4阳性单核细胞弥漫性重度间质浸润。经甲泼尼龙半脉冲疗法和泼尼松龙50mg/天治疗后，症状和实验室数据恢复正常。米库利奇病最近被报道为IgG4相关性疾病。在我们的病例中，米库利奇病合并弥漫性重度间质性肾炎经皮质类固醇成功治疗。本病例支持IgG4相关自身免疫性疾病可能是米库利奇病和间质性肾炎病因的假说。
