Case Report
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World J Gastroenterol. Dec 28, 2013; 19(48): 9490-9494
Published online Dec 28, 2013. doi: 10.3748/wjg.v19.i48.9490
IgG4-related autoimmune pancreatitis overlapping with Mikulicz’s disease and lymphadenitis: A case report
Li-Mei Qu, Ya-Hui Liu, David R Brigstock, Xiao-Yu Wen, Yong-Fang Liu, Ya-Jun Li, Run-Ping Gao
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
Ya-Hui Liu, Department of Hepatic-Biliary-Pancreatic Surgery, First Hospital, Jilin University, Changchun 130021, Jilin Province, China
David R Brigstock, The Research Institute at Nationwide Children’s Hospital, Columbus, OH 43205, United States
David R Brigstock, Division of Pediatric Surgery, Department of Surgery, The Ohio State University, Columbus, OH 43205, United States
Author contributions: Qu LM, Wen XY, Liu YH, Li YJ performed the majority of the experiments; Liu YH contributed the study design, data collection; Gao RP designed the study and wrote the manuscript; Brigstock DR co-ordinated the study and edited the manuscript; Qu LM and Liu YH contributed equally to the paper.
Supported by National Natural Scientific Foundation, No. 81070370, 81270544 (to Gao RP) and NIH 5R01AA016003 (to Brigstock D)
Correspondence to: Run-Ping Gao, Professor, Department of Hepatic-Biliary-Pancreatic Medicine, First Hospital, Jilin University, 71 Xinmin Avenue, Changchun 130021, Jilin Province, China. gao_runping@yahoo.com
Telephone: +86-431-81715110 Fax: +86-431-85612468
Received: September 15, 2013
Revised: November 16, 2013
Accepted: December 5, 2013
Published online: December 28, 2013
Abstract

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.

Keywords: IgG4-related disease, Type 1 autoimmune pancreatitis, Mikulicz’s disease, Lymphadenitis

Core tip: We report a rare case of a 63-year-old Northeast Chinese man who suffered from IgG4-related disease (RD) which involved the salivary glands, lymph node and pancreas. The patient responded promptly to prednisone therapy. Further identification and characterization of such cases is required to elucidate the prevalence and clinical features of IgG4-RD in China.