意大利多中心研究：自身免疫性胰腺炎的日常诊疗实践：临床数据、诊断、治疗及胰腺功能不全演变。
Multicentric Italian survey on daily practice for autoimmune pancreatitis: Clinical data, diagnosis, treatment, and evolution toward pancreatic insufficiency.
机构信息
Endoscopy Service, Department of Diagnostic and Therapeutic Services, IRCCS-ISMETT (Mediterranean Institute for Transplantation and Highly Specialized Therapies), Palermo, Italy.
Section of Gastroenterology, Biomedical Department of Internal and Specialized Medicine (DI.BI.M.I.S.), University of Palermo, Palermo, Italy.
出版信息
United European Gastroenterol J. 2020 Jul;8(6):705-715. doi: 10.1177/2050640620924302. Epub 2020 May 12.
BACKGROUND
Autoimmune pancreatitis (AIP) is a rare, and relatively new, form of chronic pancreatitis. The management of AIP can vary considerably among different centres in daily clinical practice.
OBJECTIVES
The aim of this study is to present a picture of epidemiological, clinical characteristics, outcomes, and the real-life practice in terms of management in several academic and non-academic centres in Italy.
METHODS
Data on the clinical presentation, diagnostic work-up, treatments, frequency of relapses, and long-term outcomes were retrospectively collected in a cohort of AIP patients diagnosed at 14 centres in Italy.
RESULTS
One hundred and six patients were classified as type 1 AIP, 48 as type 2 AIP, and 19 as not otherwise specified. Epidemiological, clinical, radiological, and serological characteristics, and relapses were similar to those previously reported for different types of AIP. Endoscopic cytohistology was available in 46.2% of cases, and diagnostic for AIP in only 35.2%. Steroid trial to aid diagnosis was administered in 43.3% cases, and effective in 93.3%. Steroid therapy was used in 70.5% of cases, and effective in 92.6% of patients. Maintenance therapy with low dose of steroid (MST) was prescribed in 25.4% of cases at a mean dose of 5 (±1.4) mg/die, and median time of MST was 60 days. Immunosuppressive drugs were rarely used (10.9%), and rituximab in 1.7%. Faecal elastase-1 was evaluated in only 31.2% of patients, and was pathological in 59.2%.
CONCLUSIONS
In this cohort of AIP patients, diagnosis and classification for subtype was frequently possible, confirming the different characteristics of AIP1 and AIP2 previously reported. Nevertheless, we observed a low use of histology and steroid trial for a diagnosis of AIP. Steroid treatment was the most used therapy in our cohort. Immunosuppressants and rituximab were rarely used. The evaluation of exocrine pancreatic insufficiency is underemployed considering its high prevalence.
背景
自身免疫性胰腺炎（AIP）是一种罕见的、相对较新的慢性胰腺炎形式。在日常临床实践中，不同中心对 AIP 的管理可能存在很大差异。
目的
本研究旨在展示意大利多个学术和非学术中心在流行病学、临床特征、结局以及管理方面的真实情况。
方法
我们回顾性收集了意大利 14 个中心诊断的 AIP 患者的临床特征、诊断检查、治疗、复发频率和长期结局的数据。
结果
106 例患者被分类为 1 型 AIP，48 例为 2 型 AIP，19 例为未分类。流行病学、临床、影像学和血清学特征以及复发与不同类型 AIP 之前报道的相似。内镜细胞组织学检查可用于 46.2%的病例，仅 35.2%的病例诊断为 AIP。43.3%的病例进行了激素试验以辅助诊断，有效率为 93.3%。70.5%的病例使用了激素治疗，有效率为 92.6%的患者。25.4%的病例使用低剂量激素维持治疗（MST），平均剂量为 5（±1.4）mg/d，MST 的中位时间为 60 天。免疫抑制剂很少使用（10.9%），利妥昔单抗为 1.7%。仅 31.2%的患者评估了粪便弹性蛋白酶-1，其中 59.2%的患者存在异常。
结论
在本队列的 AIP 患者中，诊断和亚型分类通常是可能的，证实了之前报道的 AIP1 和 AIP2 的不同特征。然而，我们观察到用于 AIP 诊断的组织学和激素试验使用率较低。在本队列中，激素治疗是最常用的治疗方法。免疫抑制剂和利妥昔单抗很少使用。考虑到外分泌胰腺功能不全的高患病率，其评估的应用不足。
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