Type 1 autoimmune pancreatitis and IgG4-related sclerosing cholangitis is associated with extrapancreatic organ failure, malignancy, and mortality in a prospective UK cohort.
Type 1 autoimmune pancreatitis and IgG4-related sclerosing cholangitis is associated with extrapancreatic organ failure, malignancy, and mortality in a prospective UK cohort.
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DOI：
10.1038/ajg.2014.223
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发表时间：
2014-10
期刊：
影响因子：
--
通讯作者：
Barnes E
中科院分区：
文献类型：
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作者：
Huggett MT;Culver EL;Kumar M;Hurst JM;Rodriguez-Justo M;Chapman MH;Johnson GJ;Pereira SP;Chapman RW;Webster GJM;Barnes E
I型自身免疫性胰腺炎（AIP）和IgG 4相关硬化性胆管炎（IgG 4相关SC）现在被认为是多系统IgG 4相关疾病（IgG 4-RD）的组成部分。我们的目的是确定从英国两个大型三级转诊中心招募的AIP/IgG 4-SC患者的临床病程和长期结局。从2004年至2013年期间确定的115例患者中收集数据，所有患者均从诊断开始进行前瞻性随访，中位时间为33个月（范围1-107），并评估对治疗的反应，多器官受累的发展和恶性肿瘤。与英国国家统计数据进行了比较。尽管97%的患者对类固醇有初步反应，但50%的患者复发。IgG 4-SC是复发的重要预测因子（P < 0.01）。11%的患者在IgG 4-RD诊断前或诊断后不久发生胰腺癌，包括3例肝胰腺胆管癌。与匹配的国家统计数据相比，诊断时或随访期间任何癌症的风险增加（比值比= 2.25，CI = 1.12-3.94，P = 0.02）。器官功能障碍发生在胰腺、肝脏、肾脏、肺和大脑中。随访期间10%的患者死亡。与匹配的国家统计数据相比，死亡风险增加（比值比= 2.07，CI = 1.07-3.55，P = 0.02）。我们的研究结果表明，AIP和IgG 4-SC与胰腺外器官衰竭和恶性肿瘤的发病率和死亡率显著相关。在首次就诊和长期随访期间，都需要对器官功能障碍和相关恶性肿瘤的证据进行详细的临床评价。
Type I autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-related SC) are now recognized as components of a multisystem IgG4-related disease (IgG4-RD). We aimed to define the clinical course and long-term outcomes in patients with AIP/IgG4-SC recruited from two large UK tertiary referral centers. Data were collected from 115 patients identified between 2004 and 2013, and all were followed up prospectively from diagnosis for a median of 33 months (range 1–107), and evaluated for response to therapy, the development of multiorgan involvement, and malignancy. Comparisons were made with national UK statistics. Although there was an initial response to steroids in 97%, relapse occurred in 50% of patients. IgG4-SC was an important predictor of relapse (P < 0.01). Malignancy occurred in 11% shortly before or after the diagnosis of IgG4-RD, including three hepatopancreaticobiliary cancers. The risk of any cancer at diagnosis or during follow-up when compared with matched national statistics was increased (odds ratio = 2.25, CI = 1.12–3.94, P = 0.02). Organ dysfunction occurred within the pancreas, liver, kidney, lung, and brain. Mortality occurred in 10% of patients during follow-up. The risk of death was increased compared with matched national statistics (odds ratio = 2.07, CI = 1.07–3.55, P = 0.02). Our findings suggest that AIP and IgG4-SC are associated with significant morbidity and mortality owing to extrapancreatic organ failure and malignancy. Detailed clinical evaluation for evidence of organ dysfunction and associated malignancy is required both at first presentation and during long-term follow-up.