Li Yanni, Zhou Lu, Zhao Xin, Song Wenjing, Karunaratna Nathasha, Wang Bangmao
Department of Gastroenterology and Hepatology, Tianjin Medical University General Hospital, Tianjin Medical University Department of Radiology, Tianjin Medical University General Hospital, Tianjin Medical University Department of Pathology, Tianjin Medical University General Hospital, Tianjin Medical University Department of Postgraduate, International School of Tianjin Medical University, Tianjin, China.
Medicine (Baltimore). 2016 Dec;95(50):e5628. doi: 10.1097/MD.0000000000005628.
While primary sclerosing cholangitis (PSC) has been recognized for decades, immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) has been correctly diagnosed only in recent years. PSC and IgG4-SC show similar clinical symptoms, serologic markers, and imaging results, but the treatment strategies and prognosis of patients differ.
Here, we present the case report of a patient diagnosed with PSC for 10 years and rediagnosed with IgG4-SC recently, to emphasize the importance of screening serum IgG4 levels in patients with previous diagnosis of PSC.
A 57-year-old woman with 10-year history of PSC was hospitalized due to pruritus. In 2004, the patient underwent cholecystectomy and cholangioenterostomy because of unexplained jaundice with pancreatic swelling. In the last 10 years, her liver enzyme levels were continuously elevated. The latest liver function profile showed elevated alanine aminotransferase, aspartate aminotransferase, and total bilirubin. IgG4 was 3.69 (0.03-2.01 g/L). Immunohistochemical staining of the surgical specimen showed >10 IgG4-positive plasma cells per high-power field, and IgG4+/IgG+ plasma cells >40%.
She was treated with prednisone 40 mg once-daily and the dose was gradually tapered. The patient remains well after 18 months.
Patients with IgG4-SC may be misdiagnosed as PSC due to lack of IgG4 screening. It is important to perform IgG4 screening in patients diagnosed as PSC. Steroid is effective to prevent disease progression in these patients.
虽然原发性硬化性胆管炎（PSC）已被认识数十年，但免疫球蛋白G4相关性硬化性胆管炎（IgG4-SC）直到近年来才得到正确诊断。PSC和IgG4-SC表现出相似的临床症状、血清学标志物和影像学结果，但患者的治疗策略和预后有所不同。
在此，我们报告一例曾被诊断为PSC 10年，近期重新诊断为IgG4-SC的患者病例，以强调对先前诊断为PSC的患者进行血清IgG4水平筛查的重要性。
一名57岁女性，有10年PSC病史，因瘙痒入院。2004年，患者因不明原因黄疸伴胰腺肿大接受了胆囊切除术和胆管肠吻合术。在过去10年中，她的肝酶水平持续升高。最新肝功能检查显示丙氨酸转氨酶、天冬氨酸转氨酶和总胆红素升高。IgG4为3.69（0.03 - 2.01g/L）。手术标本的免疫组织化学染色显示每高倍视野有>10个IgG4阳性浆细胞，且IgG4+/IgG+浆细胞>40%。
给予患者泼尼松40mg每日一次治疗，剂量逐渐递减。18个月后患者情况良好。
由于缺乏IgG4筛查，IgG4-SC患者可能被误诊为PSC。对诊断为PSC的患者进行IgG4筛查很重要。类固醇对预防这些患者的疾病进展有效。
