Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
J Dig Dis. 2019 Mar;20(3):152-158. doi: 10.1111/1751-2980.12708.
Autoimmune pancreatitis is an autoimmune disorder accompanied by clinicopathological manifestations that have been established as immunoglobulin (IgG)4-related diseases (IgG4-RD). Other IgG4-RD are often involved with autoimmune pancreatitis. They sometimes relapse despite a favorable response to steroid therapy. This study aimed to clarify the patterns and risk factors for extrapancreatic relapse.
We reviewed the data of 115 patients diagnosed with definite autoimmune pancreatitis type 1 and followed up for > 1 year. We analyzed two items: the timing and pattern of extrapancreatic relapse, and risk factors for relapse with three common manifestations: IgG4-related sclerosing cholangitis (SC), IgG4-related dacryoadenitis and sialadenitis (DS), and IgG4-related retroperitoneal fibrosis (RF).
Remission was achieved in all patients, except one. The extrapancreatic relapse rates were 11.0%, 19.7%, and 40% within 3, 5, and 10 years, respectively. Of 26 patients with extrapancreatic relapse, nine (34.6%) relapsed with a new IgG4-RD. Based on multivariate analysis, the interval between symptom onset and steroid initiation, and the presence of RF at onset were significant risk factors for relapse with SC and RF, respectively.
Our results indicate that they may be various extrapancreatic relapse patterns especially in autoimmune pancreatitis with other organ involvement. Patients with a delayed initiation of steroids or RF at onset should be carefully followed up as high-risk groups for SC and RF relapse.
自身免疫性胰腺炎是一种自身免疫性疾病，伴有已被确定为免疫球蛋白（IgG）4 相关疾病（IgG4-RD）的临床病理表现。其他 IgG4-RD 常与自身免疫性胰腺炎有关。尽管对类固醇治疗有良好反应，但它们有时会复发。本研究旨在阐明胰外复发的模式和危险因素。
我们回顾了 115 例确诊为 1 型特发性自身免疫性胰腺炎且随访时间>1 年的患者的数据。我们分析了两个项目：胰外复发的时间和模式，以及三种常见表现（IgG4 相关硬化性胆管炎（SC）、IgG4 相关泪腺和涎腺炎（DS）和 IgG4 相关腹膜后纤维化（RF））复发的危险因素。
除 1 例患者外，所有患者均达到缓解。胰外复发率分别为 3 年内、5 年内和 10 年内的 11.0%、19.7%和 40%。在 26 例胰外复发患者中，9 例（34.6%）新发 IgG4-RD 复发。基于多变量分析，症状发作与类固醇开始之间的间隔以及发病时存在 RF 是复发伴 SC 和 RF 的显著危险因素。
我们的结果表明，特别是在伴有其他器官受累的自身免疫性胰腺炎中，可能存在各种胰外复发模式。类固醇治疗开始延迟或发病时存在 RF 的患者应作为 SC 和 RF 复发的高危人群进行密切随访。
