Gong L, Shi J P
Department of Hepatology, Hangzhou Normal University Affiliated Hospital, Hangzhou 310015, China.
Zhonghua Gan Zang Bing Za Zhi. 2018 Jun 20;26(6):411-414. doi: 10.3760/cma.j.issn.1007-3418.2018.06.004.
Currently, there is no randomized controlled clinical trial of immunoglobulin (Ig) G4-related diseases in the world. Therefore, the best-known evidence-based medical treatment plan for this disorder is unavailable. The goal of IgG4-related hepatobiliary diseases treatment is to alleviate symptoms, prevent disease-related complications and fibrosis progression. A definite diagnosis is warranted before treatment. Hormonal therapy has become the basis of induction of remission in IgG4-related hepatobiliary disease. An initial prednisone dose is 30 ~ 40mg/d or 0.6 mg.kg-1.d-1 for 2 to 4 weeks, thereafter, gradually the dose is reduced within 2-3 months. Maintenance therapy with low-dose glucocorticoids hormone (prednisone 2.5 to 5.0 mg/d) is recommended for 1 to 3 years to prevent disease recurrence. In addition, immunosuppressive agents are equally effective, and in most cases, hormone combined immunosuppressive therapy may respond. Rituximab, a monoclonal antibody is a promising drug for treatment of this kind of diseases.
目前，世界上尚无关于免疫球蛋白（Ig）G4相关疾病的随机对照临床试验。因此，针对这种疾病最知名的循证医学治疗方案并不存在。IgG4相关肝胆疾病的治疗目标是缓解症状、预防疾病相关并发症和纤维化进展。治疗前需要明确诊断。激素治疗已成为IgG4相关肝胆疾病诱导缓解的基础。初始泼尼松剂量为30～40mg/d或0.6mg·kg-1·d-1，持续2至4周，此后在2至3个月内逐渐减量。建议使用低剂量糖皮质激素（泼尼松2.5至5.0mg/d）维持治疗1至3年，以预防疾病复发。此外，免疫抑制剂同样有效，在大多数情况下，激素联合免疫抑制治疗可能有效。利妥昔单抗，一种单克隆抗体，是治疗这类疾病的一种有前景的药物。
