Li-Mei Qu, Xiao-Yu Wen, Yong-Fang Liu, Ya-Jun Li, Run-Ping Gao, Department of Hepatic-Biliary-Pancreatic Medicine, First Hospital, Jilin University, Changchun 130021, Jilin Province, China.
World J Gastroenterol. 2013 Dec 28;19(48):9490-4. doi: 10.3748/wjg.v19.i48.9490.
Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis that is categorized as type 1 or type 2 according to the clinical profile. Type 1 AIP, which predominantly presents in a few Asian countries, is a hyper-IgG4-related disease. We report a case of IgG4-related AIP overlapping with Mikulicz's disease and lymphadenitis, which is rare and seldom reported in literature. A 63-year male from Northeast China was admitted for abdominal distension lasting for one year. He presented symmetric swelling of the parotid and submandibular glands with slight dysfunction of salivary secretion for 6 mo. He had a 2-year history of bilateral submandibular lymphadenopathy without pain. He underwent surgical excision of the right submandibular lymph node one year prior to admission. He denied any history of alcohol, tobacco, or illicit drug use. Serological examination revealed high fasting blood sugar level (8.8 mmol/L) and high level of IgG4 (15.2 g/L). Anti-SSA or anti-SSB were negative. Computed tomography of the abdomen showed a diffusely enlarged pancreas with loss of lobulation. Immunohistochemical stain for IgG4 demonstrated diffuse infiltration of IgG4-positive plasma cells in labial salivary gland and lymph node biopsy specimens. The patient received a dose of 30 mg/d of prednisone for three weeks. At this three-week follow-up, the patient reported no discomfort and his swollen salivary glands, neck lymph node and pancreas had returned to normal size. The patient received a maintenance dose of 10 mg/d of prednisone for 6 mo, after which his illness had not recurred.
自身免疫性胰腺炎（AIP）是一种慢性胰腺炎，根据临床表现可分为 1 型或 2 型。主要在少数亚洲国家出现的 1 型 AIP 是一种高 IgG4 相关疾病。我们报告了一例 IgG4 相关 AIP 重叠米库利茨病和淋巴结炎的病例，这种情况很少见，文献中也很少报道。一名来自中国东北的 63 岁男性因腹胀持续一年入院。他表现为双侧腮腺和颌下腺对称性肿胀，唾液分泌轻度功能障碍 6 个月。他有双侧颌下淋巴结肿大 2 年，无疼痛。入院前一年，他接受了右侧颌下淋巴结切除术。他否认有饮酒、吸烟或使用非法药物的病史。血清学检查显示空腹血糖水平升高（8.8 mmol/L）和 IgG4 水平升高（15.2 g/L）。抗 SSA 或抗 SSB 为阴性。腹部计算机断层扫描显示胰腺弥漫性肿大，失去分叶。唇腺和淋巴结活检标本的 IgG4 免疫组织化学染色显示 IgG4 阳性浆细胞弥漫浸润。患者接受了三周剂量为 30 mg/d 的泼尼松治疗。在这三周的随访中，患者报告没有不适，他肿胀的唾液腺、颈部淋巴结和胰腺已恢复正常大小。患者接受了 6 个月剂量为 10 mg/d 的泼尼松维持治疗，此后病情未再复发。
