Department of Internal Medicine and Hepatogastroenterology, University Hospital Brno, Masaryk University Faculty of Medicine, Brno, Czech Republic. ; Department of Internal Medicine, University of Ostrava Faculty of Medicine, Ostrava, Czech Republic.
Gut Liver. 2013 Sep;7(5):621-4. doi: 10.5009/gnl.2013.7.5.621. Epub 2013 Sep 11.
Autoimmune pancreatitis (AIP) type 1 is commonly associated with simultaneous involvement of extrapancreatic organs. Sclerosing cholangitis, sialadenitis, retroperitoneal fibrosis, Sjögren syndrome, and other extrapancreatic lesions are often observed concurrently with AIP. High levels of immunoglobulin G4 (IgG4) in the blood serum and affected tissues are typical of this diagnostic entity. We describe a case report of a 58-year-old female with findings of AIP (according to Asian criteria), IgG4-positive mastitis, and histologically verified Mikulicz syndrome. The effect of corticoid therapy supported the diagnosis of AIP and simultaneously led to the eradication of recurrent mastitis. To the best of our knowledge, this is the first reported case of concurrent findings of AIP and IgG4 mastitis. Our case report supports the concept of systemic IgG4 syndrome with multisystem involvement. Timely diagnosis and appropriate therapy can be effective in a high percentage of patients.
自身免疫性胰腺炎（AIP）1 型通常与胰腺外器官同时受累有关。硬化性胆管炎、唾液腺炎、腹膜后纤维化、干燥综合征和其他胰腺外病变常与 AIP 同时发生。血清和受累组织中免疫球蛋白 G4（IgG4）水平升高是该诊断实体的典型特征。我们描述了一例 58 岁女性的病例报告，其表现为 AIP（根据亚洲标准）、IgG4 阳性乳腺炎和组织学证实的 Mikulicz 综合征。皮质类固醇治疗的效果支持了 AIP 的诊断，并同时根除了复发性乳腺炎。据我们所知，这是首例同时发现 AIP 和 IgG4 乳腺炎的病例报告。我们的病例报告支持多系统受累的全身性 IgG4 综合征的概念。及时诊断和适当治疗在很大比例的患者中是有效的。
