Ohno Yoshinori, Kumagi Teru, Yokota Tomoyuki, Azemoto Nobuaki, Tanaka Yoshinori, Tange Kazuhiro, Inada Nobu, Miyata Hideki, Imamura Yoshiki, Koizumi Mitsuhito, Kuroda Taira, Hiasa Yoichi
Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime, 791-0295, Japan.
Center for Liver-Biliary-Pancreatic Diseases, Matsuyama Red Cross Hospital, Matsuyama, 790-8524, Ehime, Japan.
Orphanet J Rare Dis. 2016 Jul 28;11(1):103. doi: 10.1186/s13023-016-0487-y.
Type 1 autoimmune pancreatitis (AIP) is clinically characterized by a response to steroid therapy. Despite having a favorable prognosis, AIP has a high relapse rate and factors predicting relapse in AIP patients treated with steroids have not yet been established.
A retrospective chart review was conducted of 32 newly diagnosed type 1 AIP patients who had undergone enhanced computed tomography (CT) pre- and post-steroid therapy.
Ten patients experienced relapse. Pancreatic volume was reduced significantly in all patients (pre-treatment volume, 88.5 ± 32.9 cm(3) vs. post-treatment volume, 45.4 ± 21.1 cm(3); P < 0.001), although the pre-treatment pancreatic volume did not differ between the relapse and non-relapse groups (92.6 ± 10.5 cm(3) vs. 86.6 ± 7.1 cm(3), P = 0.401). However, the post-treatment pancreatic volume was significantly greater in the relapse group than that in the non-relapse group (56.9 ± 6.3 cm(3) vs. 40.2 ± 4.2 cm(3), P = 0.008). Similarly, the percent reduction in pancreatic volume was significantly smaller in the relapse group than that in the non-relapse group (36.6 ± 4.7 % vs. 52.1 ± 3.2 %, P = 0.002). Multivariate analysis identified post-treatment pancreatic volume (HR, 1.04, 95 % CI: 1.01-1.08, P = 0.010) and percent reduction in pancreatic volume (HR, 0.87, 95 % CI: 0.79-0.94, P < 0.001) as predictive factors for relapse of type 1 AIP. A post-treatment pancreatic volume of 50 cm(3) < (P = 0.009) and a percent reduction in the pancreatic volume of <35 % (P = 0.004) had a significantly high relapse rate. These data suggest that early pancreatic volume changes after steroid therapy may be a useful prognostic value, because type 1 AIP patients with a high post-treatment pancreatic volume or low pancreatic volume reduction showed significant relapse.
Early pancreatic volume reduction on CT after steroid therapy indicates the therapeutic effects of steroids, and a low decrease in the pancreatic volume may be associated with a limited response that predicts future relapse in patients with type 1 AIP. Reduction of steroids in these cases must be observed carefully with consideration of immunomodulator use.
1型自身免疫性胰腺炎（AIP）的临床特征是对类固醇治疗有反应。尽管预后良好，但AIP的复发率很高，且尚未确定预测接受类固醇治疗的AIP患者复发的因素。
对32例新诊断的1型AIP患者进行回顾性病历审查，这些患者在类固醇治疗前后均接受了增强计算机断层扫描（CT）。
10例患者复发。所有患者的胰腺体积均显著减小（治疗前体积，88.5±32.9 cm³ vs. 治疗后体积，45.4±21.1 cm³；P<0.001），尽管复发组和未复发组的治疗前胰腺体积无差异（92.6±10.5 cm³ vs. 86.6±7.1 cm³，P=0.401）。然而，复发组的治疗后胰腺体积显著大于未复发组（56.9±6.3 cm³ vs. 40.2±4.2 cm³，P=0.008）。同样，复发组胰腺体积减少百分比显著低于未复发组（36.6±4.7% vs. 52.1±3.2%，P=0.002）。多变量分析确定治疗后胰腺体积（HR，1.04，95%CI：1.01-1.08，P=0.010）和胰腺体积减少百分比（HR，0.87，95%CI：0.79-0.94，P<0.001）为1型AIP复发的预测因素。治疗后胰腺体积>50 cm³（P=0.009）和胰腺体积减少百分比<35%（P=0.004）的复发率显著较高。这些数据表明，类固醇治疗后早期胰腺体积变化可能具有有用的预后价值，因为治疗后胰腺体积高或胰腺体积减少低的1型AIP患者显示出显著复发。
类固醇治疗后CT上早期胰腺体积减小表明类固醇的治疗效果，胰腺体积降低幅度小可能与反应有限有关，这预示着1型AIP患者未来会复发。在这些情况下，必须谨慎观察类固醇的减量，并考虑使用免疫调节剂。
