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.

中文翻译：

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意大利对自身免疫性胰腺炎日常实践的多中心调查：临床数据，诊断，治疗以及向胰腺功能不全的演变。

背景技术自身免疫性胰腺炎（AIP）是一种罕见且相对较新的慢性胰腺炎。在日常临床实践中，不同中心之间的AIP管理可能存在很大差异。目的本研究的目的是展示意大利几个学术和非学术中心在管理方面的流行病学，临床特征，结局和现实生活实践情况。方法回顾性收集意大利14个中心确诊的AIP患者队列的临床表现，诊断检查，治疗，复发频率和长期结果的数据。结果106例患者被分类为1型AIP，48例为2型AIP，19例未另行说明。流行病学，临床，放射学和血清学特征，复发与以前报道的不同类型的AIP相似。内镜下的细胞组织学检查占46.2％，诊断为AIP的仅占35.2％。进行类固醇激素试验以帮助诊断的病例为43.3％，有效率为93.3％。类固醇疗法占70.5％，有效率为92.6％。在25.4％的病例中开出了低剂量类固醇（MST）维持治疗的建议，平均剂量为5（±1.4）mg / die，MST的中位时间为60天。很少使用免疫抑制药物（占10.9％），而利妥昔单抗占1.7％。仅在31.2％的患者中评估了粪便弹性蛋白酶1，在59.2％的患者中进行了病理检查。结论在这一AIP患者队列中，亚型的诊断和分类常常是可能的，证实了先前报道的AIP1和AIP2的不同特征。然而，我们观察到组织学和类固醇试验在诊断AIP方面的使用率较低。类固醇治疗是我们队列中最常用的治疗方法。很少使用免疫抑制剂和利妥昔单抗。考虑到外分泌型胰腺功能不全的患病率高，对其评估尚不充分。