摘要
背景IgG4相关性自身免疫性胰腺炎(IgG4 related autoim-mune pancreatitis,IgG4-AIP)是一种由自身免疫介导的胰腺炎,该病的临床表现缺乏特异性,鉴别诊断常有难度,糖皮质激素对该疾病的治疗的指征及疗程仍存争议.本研究希望通过分析疾病的临床特征提高疾病的认识和诊疗,通过分析疾病复发的相关影响因素找出降低疾病复发率的治疗方案.目的研究IgG4-AIP的临床特征及其治疗疗效、复发的影响因素.方法回顾性分析46例确诊IgG4-AIP患者的临床资料特点及分析疾病复发的相关影响因素.结果本研究中,纳入的46例患者IgG4-AIP患者男女比例为10.5:1.IgG4-AIP最常见的临床表现为梗阻性黄疸.52.1%的IgG4-AIP患者合并了胰外器官受累,最常见的胰外器官为胆管.91.3%的IgG4-AIP患者血清IgG4数值高于正常值.IgG4-AIP的影像学检查主要表现为胰腺弥漫肿胀或局部肿大,伴胆胰管狭窄.81.2%的IgG4-AIP患者通过超声内镜引导下胰腺穿刺活检获得病理学组织,典型的IgG4-AIP病理学检查示胰腺纤维组织增生,淋巴细胞、浆细胞浸润,胰腺腺泡萎缩,其中50%的IgG4-AIP患者病理免疫组化法示大量IgG4阳性浆细胞.糖皮质激素治疗IgG4-AIP均有效,但36.8%的患者治疗缓解后复发,其中71.4%患者是停用糖皮质激素后复发.Logistic多因素回归分析示复发的危险因素是胰源性门脉高压和胰外器官受累,小剂量糖皮质激素长期维持治疗可以预防复发.结论IgG4-AIP的临床表现缺乏特异性,容易被误诊为胰腺癌.IgG4-AIP的主要表现为血清IgG4水平升高、胰腺弥漫肿胀或局部肿大伴胆胰管狭窄、多器官受累、IgG4阳性浆细胞浸润、组织纤维化、对糖皮质激素治疗有效.IgG4-AIP复发的危险因素是胰源性门脉高压和胰外器官受累,小剂量糖皮质激素维持治疗可以预防复发.
BACKGROUND IgG4-associated autoimmune pancreatitis(IgG4-AIP)is a kind of autoimmune-mediated pancreatitis.The clinical manifestations of IgG4-AIP are not specific and the differential diagnosis is often difficult,and the indications and course of glucocorticoid treatment for the disease are still controversial.The aim of this study was to improve the understanding,diagnosis,and treatment of the disease by analyzing the clinical characteristics of the disease,and to reduce the recurrence rate of the disease by analyzing the factors related to its recurrence.AIM To investigate the clinical features,therapeutic efficacy,and recurrence of IgG4-AIP.METHODS The clinical data of 46 patients with IgG4-AIP were analyzed retrospectively.RESULTS The male to female ratio of IgG4-AIP patients was 10.5:1.The most common clinical manifestation was obstructive jaundice.Approximately 52.1%of patients had extrapancreatic organ involvement,with the most commonly affected extrapancreatic organ being the bile duct.The vast majority(91.3%)of patients had higher serum IgG4 levels than normal.The imaging findings were diffuse swelling or local enlargement of the pancreas with stricture of the biliary and pancreatic ducts.The majority(81.2%)of patients underwent pancreatic biopsy under the guidance of endoscopic ultrasonography.Typical pathological findings included fibrous tissue proliferation,lymphocyte and plasma cell infiltration,and acinar atrophy of the pancreas.Half of the patients showed a large number of IgG4 positive plasma cells as revealed by immunohistochemistry.Glucocorticoids were effective for IgG4-AIP,but 36.8%of the patients relapsed after remission,and 71.4%relapsed after stopping glucocorticoids.Logistic regression analysis showed that the risk factors for recurrence were pancreatic portal hypertension and involvement of extra-pancreatic organs.Low-dose glucocorticoid maintenance therapy could prevent recurrence.CONCLUSION Due to the lack of specific clinical manifestation,IgG4-AIP is easily misdiagnosed as pancreatic
作者
曹卉
曹仕琼
高雨彤
杜凡
Hui Cao;Shi-Qiong Cao;Yu-Tong Gao;Fan Du(Department of Gastroenterology,Liyuan Hospital of Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430066,Hubei Province,China;Department of Pathology,Union Hospital of Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430022,Hubei Province,China;Department of Gastroenterology,Union Hospital of Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430022,Hubei Province,China)
出处
《世界华人消化杂志》
CAS
2023年第16期677-687,共11页
World Chinese Journal of Digestology
基金
国家自然科学基金青年基金项目,No.81700471.
