Huggett Matthew T, Culver E L, Kumar M, Hurst J M, Rodriguez-Justo M, Chapman M H, Johnson G J, Pereira S P, Chapman R W, Webster George J M, Barnes E
UCL Institute for Liver and Digestive Health, University College London, London, UK.
Department of Gastroenterology and Hepatology, University College Hospital, London, UK.
Am J Gastroenterol. 2014 Oct;109(10):1675-1683. doi: 10.1038/ajg.2014.223. Epub 2014 Aug 26.
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.
I型自身免疫性胰腺炎（AIP）和IgG4相关性硬化性胆管炎（IgG4相关SC）现被认为是多系统IgG4相关性疾病（IgG4-RD）的组成部分。我们旨在明确从英国两家大型三级转诊中心招募的AIP/IgG4-SC患者的临床病程和长期预后。
收集了2004年至2013年间确诊的115例患者的数据，所有患者自诊断起进行前瞻性随访，中位随访时间为33个月（范围1-107个月），评估治疗反应、多器官受累情况及恶性肿瘤发生情况。与英国国家统计数据进行比较。
尽管97%的患者最初对类固醇治疗有反应，但50%的患者出现复发。IgG4-SC是复发的重要预测因素（P<0.01）。在IgG4-RD诊断前后不久，11%的患者发生恶性肿瘤，其中包括3例肝胰胆管癌。与匹配的英国国家统计数据相比，诊断时或随访期间发生任何癌症的风险增加（比值比=2.25，可信区间=1.12-3.94，P=0.02）。胰腺、肝脏、肾脏、肺和脑均出现器官功能障碍。随访期间10%的患者死亡。与匹配的英国国家统计数据相比，死亡风险增加（比值比=2.07，可信区间=1.07-3.55，P=0.02）。
我们的研究结果表明，AIP和IgG4-SC由于胰腺外器官衰竭和恶性肿瘤而与显著的发病率和死亡率相关。在初次就诊时和长期随访期间，都需要进行详细的临床评估，以寻找器官功能障碍和相关恶性肿瘤的证据。
