特发性胰腺炎NOS：被遗忘类型的临床特征和结局。
Autoimmune pancreatitis not otherwise specified (NOS): Clinical features and outcomes of the forgotten type.
机构信息
Gastroenterology Unit, Pancreas Center, University of Verona, Piazzale L.A. Scuro 10, 37134, Verona, Italy.
Gastroenterology Unit, Pancreas Center, University of Verona, Piazzale L.A. Scuro 10, 37134, Verona, Italy.
出版信息
Hepatobiliary Pancreat Dis Int. 2019 Dec;18(6):576-579. doi: 10.1016/j.hbpd.2019.05.010. Epub 2019 Jun 11.
BACKGROUND
Autoimmune pancreatitis (AIP) is a well-recognized fibroinflammatory disease of the pancreas. Despite the significant number of studies published on AIP type 1 and 2, no studies have been focused on AIP type not otherwise specified (NOS) and therefore very little is known about clinical features and long-term outcomes of these patients. The aim of this study was to investigate clinical and radiological features of AIP type NOS-patients.
METHODS
Patients classified as AIP type NOS at clinical onset included in our database prospectively maintained since 1995 were evaluated. Epidemiological, clinical data were collected and analyzed.
RESULTS
Forty-six patients were included in the study. The clinical onset was mainly characterized by weight loss, jaundice and acute pancreatitis. Eight patients (17.4%) were reclassified as AIP type 2 during follow-up because of the development of ulcerative colitis. Seven patients (15.2%) experienced relapse after steroid treatment but only one (2.2%) needed immunosuppressive drugs because of recurrent relapses.
CONCLUSIONS
AIP type NOS shares clinical features similar to AIP type 2 and a relevant proportion of patients was reclassified as AIP type 2 during follow-up because of the development of ulcerative colitis. The risk of relapse is low but not irrelevant.
背景
自身免疫性胰腺炎（AIP）是一种公认的胰腺纤维炎症性疾病。尽管已经发表了大量关于 AIP 1 型和 2 型的研究，但尚未有研究聚焦于未特指型（NOS）AIP，因此，对于这些患者的临床特征和长期结局知之甚少。本研究旨在探讨 NOS 型 AIP 患者的临床和影像学特征。
方法
我们对自 1995 年以来前瞻性纳入数据库的NOS 型 AIP 患者进行了评估，收集了流行病学和临床数据并进行了分析。
结果
本研究共纳入 46 例患者。临床起病主要表现为体重减轻、黄疸和急性胰腺炎。8 例（17.4%）患者在随访期间因溃疡性结肠炎的发生而重新归类为 AIP 2 型。7 例（15.2%）患者在类固醇治疗后复发，但仅有 1 例（2.2%）因反复复发而需要免疫抑制治疗。
结论
NOS 型 AIP 与 AIP 2 型具有相似的临床特征，且在随访过程中有相当一部分患者因溃疡性结肠炎的发生而重新归类为 AIP 2 型。复发风险较低，但并非无关紧要。
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