Mittelstaedt Anke, Meier Peter N, Dankoweit-Timpe Eva, Christ Beate, Jaehne Joachim
Clinic for General and Digestive Surgery, Center for Endocrine, Oncologic and Metabolic Surgery, DIAKOVERE Henriettenstift, Marienstraße 72-90, D-30171 Hannover, Germany.
Clinic for Gastroenterology, DIAKOVERE Henriettenstift, Marienstraße 72-90, D-30171 Hannover, Germany.
Innov Surg Sci. 2018 May 25;3(2):157-163. doi: 10.1515/iss-2018-0010. eCollection 2018 Jun.
Even though IgG4-related disease has gained increased attention worldwide, the diagnosis remains challenging. IgG4-related sclerosing cholangitis (IgG4-SC) is not well described in the western hemisphere and may mimic cholangiocarcinoma (CC), especially when occurring without other symptoms such as, e.g. concurrent pancreatitis or retroperitoneal fibrosis. We present a case to add further information to the diagnosis and treatment of this challenging disease.
A 60-year-old male patient presented with painless jaundice. Prior medical history showed diabetes mellitus type I, high blood pressure, and deep vein thrombosis. Diagnostic investigations were strongly suspicious of a Klatskin tumor, although biopsies were inconclusive. The tumor marker Carbohydrate Antigen 19-9 (CA 19-9) was elevated. Prior to the recommended surgery, the patient had two second opinions in two different university hospitals, both arguing for surgery as well. The patient received hilar resection with right hemihepatectomy. During the postoperative course, some major complications occurred, i.e. recurrent pleural effusion, abscess in the liver resection area, sepsis, ileus, and restricted liver metabolism. Treatment with prednisolone did not show any improvement. Approximately 3 months after surgery, the patient died in consequence of acute respiratory failure. Histology showed no signs of CC, but IgG4-SC could be diagnosed.
In the case of preoperative signs of CC, differential diagnosis of IgG4-SC needs to be considered, in particular, in cases with missing histologic proof of malignant disease.
尽管IgG4相关性疾病在全球范围内受到越来越多的关注，但其诊断仍然具有挑战性。IgG4相关性硬化性胆管炎（IgG4-SC）在西半球的描述并不充分，可能会被误诊为胆管癌（CC），尤其是在没有并发胰腺炎或腹膜后纤维化等其他症状的情况下。我们报告一例病例，以进一步补充有关这种具有挑战性疾病的诊断和治疗的信息。
一名60岁男性患者出现无痛性黄疸。既往病史显示患有I型糖尿病、高血压和深静脉血栓形成。诊断性检查强烈怀疑为肝门部肿瘤，尽管活检结果不明确。肿瘤标志物糖类抗原19-9（CA 19-9）升高。在建议的手术前，患者在两家不同的大学医院获得了另外两种意见，两者也都主张进行手术。患者接受了肝门部切除及右半肝切除术。术后过程中出现了一些严重并发症，即反复胸腔积液、肝切除区域脓肿、败血症、肠梗阻和肝脏代谢受限。泼尼松龙治疗未见任何改善。手术后约3个月，患者因急性呼吸衰竭死亡。组织学检查未显示CC迹象，但可诊断为IgG4-SC。
对于术前有CC迹象的病例，尤其是在缺乏恶性疾病组织学证据的情况下，需要考虑IgG4-SC的鉴别诊断。
