免疫球蛋白 IgG4 相关硬化性胆管炎的临床特征：大样本中伴有和不伴有自身免疫性胰腺炎病例的比较。
Clinical characteristics of immunoglobulin IgG4-related sclerosing cholangitis: Comparison of cases with and without autoimmune pancreatitis in a large cohort.
机构信息
Department of Gastroenterology, Nagoya City University Graduate School of Medical Sciences, 1 Kawasumi, Mizuho-cho, Mizuho-ku Nagoya 467-8601, Japan.
Department of Internal Medicine, Tokyo Metropolitan, Komagome Hospital, Tokyo, Japan.
出版信息
Dig Liver Dis. 2021 Oct;53(10):1308-1314. doi: 10.1016/j.dld.2021.02.009. Epub 2021 Mar 2.
BACKGROUND
The clinical characteristics of IgG4-related sclerosing cholangitis (IgG4-SC) especially without autoimmune pancreatitis (AIP) have not been investigated in a large cohort.
AIMS
To clarify the clinical characteristics of IgG4-SC and IgG4-SC without AIP.
METHODS
We retrospectively reviewed imaging, serology, other organ involvement (OOI) and histology of 872 patients with IgG4-SC who participated in a Japanese nationwide survey in 2019, and compared these items between IgG4-SC with and without AIP.
RESULTS
AIP was present in 83.7% (730/872) of IgG4-SC. In IgG4-SC, bile duct wall thickening was observed on ultrasound (528/650; 81.2%), computed tomography (375/525; 71.4%) and magnetic resonance imaging or cholangiopancreatography (290/440; 65.9%). An elevated serum IgG4 level (≥ 135 mg/dL) was found in 88.0% (322/366). IgG4-related OOI other than AIP was observed in 25.2% (211/836). The proportion of females was significantly higher in IgG4-SC without AIP (28.9% vs. 20.1%; p = 0.025). Hilar stricture was the most common cholangiographic type in IgG4-SC without AIP (39/107; 36.4%).There were no significant differences between IgG4-SC with and without AIP in the rates of bile duct wall thickening, elevated serum IgG4 level, or IgG4-related OOI.
CONCLUSIONS
The clinical characteristics of IgG4-SC was similar between IgG4-SC with and without AIP in a large cohort.
背景
尚未在大规模队列中研究 IgG4 相关硬化性胆管炎（IgG4-SC），尤其是不伴有自身免疫性胰腺炎（AIP）的 IgG4-SC 的临床特征。
目的
阐明 IgG4-SC 和不伴有 AIP 的 IgG4-SC 的临床特征。
方法
我们回顾性分析了 2019 年参加日本全国性调查的 872 例 IgG4-SC 患者的影像学、血清学、其他器官受累（OOI）和组织学资料，并比较了伴有和不伴有 AIP 的 IgG4-SC 患者的这些项目。
结果
83.7%（730/872）的 IgG4-SC 患者伴有 AIP。在 IgG4-SC 中，超声（528/650；81.2%）、计算机断层扫描（375/525；71.4%）和磁共振成像或胆管胰管造影（290/440；65.9%）检查可见胆管壁增厚。88.0%（322/366）患者血清 IgG4 水平升高（≥135mg/dL）。除 AIP 以外 IgG4 相关的 OOI 见于 25.2%（211/836）患者。不伴有 AIP 的 IgG4-SC 患者中女性比例显著高于伴有 AIP 的 IgG4-SC 患者（28.9%比 20.1%；p=0.025）。不伴有 AIP 的 IgG4-SC 患者中最常见的胆管造影类型是肝门部狭窄（39/107；36.4%）。伴有和不伴有 AIP 的 IgG4-SC 患者中胆管壁增厚、血清 IgG4 水平升高或 IgG4 相关 OOI 的发生率均无显著差异。
结论
在大规模队列中，伴有和不伴有 AIP 的 IgG4-SC 的临床特征相似。
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