El Euch Mounira, Hddad Souha, Mahfoudhi Madiha, Maktouf Hela, Ben Hamida Fethi, Jaziri Fatima, Ben Abdelghani Khaoula, Turki Sami, Ben Abdallah Taïeb
Department of Internal Medicine 'A', Charles Nicolle Hospital, Tunis, Tunisia.
University of Tunis Manar, Tunis, Tunisia.
Am J Case Rep. 2017 Jul 24;18:822-825. doi: 10.12659/ajcr.904263.
BACKGROUND Type 1 autoimmune pancreatitis (AIP), also known as lymphoplasmacytic sclerosing pancreatitis (LPSP), is a rare cause of chronic pancreatitis, characterized by a fibro-inflammatory process. However, patients with AIP may have a good response to corticosteroid therapy. We describe a Tunisian patient with AIP that was confirmed to be an IgG4-related disease (IgG4-RD). CASE REPORT We describe a case of a 70-year-old man who was admitted to hospital for obstructive jaundice and abdominal pain. Serum liver function tests were abnormal and upper abdominal computed tomography (CT) imaging showed diffuse pancreatic swelling and strictures of the main pancreatic duct without any focal lesion. Pancreatico-biliary magnetic resonance imaging (MRI) showed a thickened rim surrounding the pancreatic duct Serum IgG4 levels were elevated, resulting in a diagnosis of IgG4-related AIP. The patient showed a good clinical, biochemical, and radiological response following steroid therapy in combination with azathioprine. CONCLUSIONS The diagnostic workup of IgG4-RD is complex and usually requires a combination of clinical examination, imaging, and serological analysis. As this case report has demonstrated, IgG4-RD should be considered in patients who present with pancreatitis or AIP, because of the favorable response to steroid therapy, particularly when treatment is initiated early.
背景 1 型自身免疫性胰腺炎（AIP），也称为淋巴细胞浆细胞性硬化性胰腺炎（LPSP），是慢性胰腺炎的一种罕见病因，其特征为纤维炎症过程。然而，AIP 患者对皮质类固醇治疗可能有良好反应。我们描述了一名突尼斯 AIP 患者，该患者被确诊为 IgG4 相关疾病（IgG4-RD）。病例报告 我们描述了一例 70 岁男性患者，因梗阻性黄疸和腹痛入院。血清肝功能检查异常，上腹部计算机断层扫描（CT）成像显示胰腺弥漫性肿胀和主胰管狭窄，无任何局灶性病变。胰胆管磁共振成像（MRI）显示胰管周围有增厚的边缘。血清 IgG4 水平升高，从而诊断为 IgG4 相关 AIP。该患者在接受类固醇治疗联合硫唑嘌呤后，临床、生化和影像学方面均显示出良好反应。结论 IgG4-RD 的诊断检查很复杂，通常需要结合临床检查、影像学和血清学分析。正如本病例报告所示，对于出现胰腺炎或 AIP 的患者，应考虑 IgG4-RD，因为其对类固醇治疗反应良好，尤其是在早期开始治疗时。
