Abstract：
BACKGROUND Systemic IgG4-related disease is a rare disease that can affect the hepatobiliary system and may lead to tissue fibrosis and organ failure. Diagnostic criteria for IgG4-related disease are well established, and systemic glucocorticoids are recommended for initiation of treatment. Besides the beneficial properties of glucocorticoids, the long-term treatment with systemic steroids carries the risk of toxicity, especially in elderly patients, in whom IgG4-related disease is more common. Furthermore, disease relapses may occur during the tapering of steroids. Overall, the optimal treatment approach for maintenance therapy has not been clarified yet and is an area of current clinical research. CASE REPORT We present a patient with IgG4-related sclerosing cholangitis and histologically confirmed systemic (multi-organ) IgG4-related disease who was at increased risk of disease recurrence. The effects of immunosuppressants (prednisolone, 6-mercaptopurine, budesonide) on clinical symptoms, laboratory parameters (AST, ALT, AP, γGT, bilirubin), and imaging examinations (magnetic resonance cholangiography) were documented over 56 months. Control of IgG4-related sclerosing cholangitis was achieved - without systemic prednisolone - with the locally acting glucocorticoid budesonide in combination with low-dose 6-mercaptopurine. During treatment with 6-mercaptopurine, transient hepatotoxicity occurred, which was reversed by intermittent pausing and subsequent dose reduction. In addition, gangrenous cholecystitis occurred as a complication of immunosuppression and was treated by emergency cholecystectomy. CONCLUSIONS Budesonide could be a new treatment modality for IgG4-related sclerosing cholangitis. Systemic manifestations of immunoglobulin G4-related disease can be controlled with low-dose 6-mercaptopurine. Gangrenous cholecystitis may occur as a complication of immunosuppressive treatment.
摘要：
背景技术系统性IgG4相关疾病是一种罕见的疾病,其可影响肝胆系统并可导致组织纤维化和器官衰竭。IgG4相关疾病的诊断标准已经确立，和全身性糖皮质激素建议开始治疗。除了糖皮质激素的有益性质，全身性类固醇的长期治疗具有毒性的风险，尤其是老年患者，其中IgG4相关疾病更常见。此外，在类固醇逐渐减少期间，疾病可能会复发。总的来说,维持治疗的最佳治疗方法尚未明确，这是当前临床研究的一个领域。病例报告我们介绍了1例IgG4相关硬化性胆管炎和组织学证实的全身性（多器官）IgG4相关疾病的患者，其疾病复发风险增加。免疫抑制剂（泼尼松龙，6-巯基嘌呤,布地奈德）对临床症状，实验室参数(AST，ALT,AP,γGT,胆红素），和影像学检查(磁共振胆管造影)记录超过56个月.在没有全身性泼尼松龙的情况下，使用局部作用的糖皮质激素布地奈德与低剂量6-巯基嘌呤联合使用，可以控制IgG4相关的硬化性胆管炎。在用6-巯基嘌呤治疗期间，发生了短暂的肝毒性，通过间歇性暂停和随后的剂量减少而逆转。此外,坏疽性胆囊炎是免疫抑制的并发症，并通过紧急胆囊切除术治疗。结论布地奈德可能是IgG4相关硬化性胆管炎的一种新的治疗方式。免疫球蛋白G4相关疾病的全身表现可以用低剂量6-巯基嘌呤控制。坏疽性胆囊炎可能是免疫抑制治疗的并发症。
