Department of Gastroenterology, Hepatology and Transplant, Lady Cilento Children's Hospital.
Faculty of Medicine, University of Queensland, Brisbane, Australia.
J Pediatr Gastroenterol Nutr. 2018 Jul;67(1):80-85. doi: 10.1097/MPG.0000000000001927.
Autoimmune liver disease (AILD) incorporates primary sclerosing cholangitis (PSC), autoimmune hepatitis (AIH), and autoimmune sclerosing cholangitis (ASC). ASC is a condition that includes overlap of AIH and PSC. We investigate changes in practice in relation to diagnosis and phenotype over 2 time periods.
Retrospective chart review was conducted from January 2000 to 2016. Data were divided into two 8-year cohorts, CI and C2.
Data were collected in 75 children, 29 in 2000-2007 (C1) and 46 in 2008-2016 (C2). Presenting AILD type was AIH in 59%, ASC in 10%, and PSC in 31%. Final AILD type was AIH in 53%, ASC in 16%, and PSC in 31%. When comparing C1 to C2, those with AIH decreased (65% vs 45%) and those with ASC increased (14% vs 18%). Use of magnetic resonance cholangio-pancreatography increased from 34% in C1 to 65% in C2. Advanced liver disease on biopsy was noted in 53% of all children at presentation. Only 5 female children progressed to liver transplant (3 ASC-IBD [inflammatory bowel disease]; 1 PSC-IBD; 1 AIH). Colonoscopy performance increased from 48% in C1 to 63% in C2 with diagnosis of AILD-IBD increasing from 31% to 52%. Right-sided disease was present in 46% and macroscopic rectal sparing in 36% of those with ulcerative colitis (UC). Colectomy was required in 3 children with large duct PSC-IBD.
PSC and ASC are increasing in relevance along with IBD and reflect increasing performance of magnetic resonance cholangio-pancreatography and colonoscopy. Large duct PSC and ASC with IBD are risk factors for colectomy and along with female gender, for liver transplant.
自身免疫性肝病（AILD）包括原发性硬化性胆管炎（PSC）、自身免疫性肝炎（AIH）和自身免疫性硬化性胆管炎（ASC）。ASC 是一种重叠 AIH 和 PSC 的疾病。我们调查了在两个时间段内与诊断和表型相关的实践变化。
回顾性病历审查于 2000 年 1 月至 2016 年进行。数据分为两个 8 年队列，CI 和 C2。
共收集 75 名儿童的数据，其中 2000-2007 年（C1）为 29 例，2008-2016 年（C2）为 46 例。表现为 AILD 类型的患者中，AIH 占 59%，ASC 占 10%，PSC 占 31%。最终 AILD 类型为 AIH 占 53%，ASC 占 16%，PSC 占 31%。与 C1 相比，C2 中 AIH 患者减少（65%对 45%），ASC 患者增加（14%对 18%）。磁共振胆胰管造影的使用率从 C1 的 34%增加到 C2 的 65%。所有患儿初诊时均有 53%存在进展性肝病史。仅有 5 名女性患儿进展为肝移植（3 例 ASC-IBD[炎症性肠病]；1 例 PSC-IBD；1 例 AIH）。从 C1 的 48%增加到 C2 的 63%，与 AILD-IBD 的诊断从 31%增加到 52%，结肠镜检查的应用增加。溃疡性结肠炎（UC）患者中，右半结肠炎占 46%，直肠大体保留占 36%。3 例伴有大胆管 PSC-IBD 的患儿需要结肠切除术。
PSC 和 ASC 与 IBD 一起的相关性增加，反映了磁共振胆胰管造影和结肠镜检查的应用增加。伴有 IBD 的大胆管 PSC 和 ASC 是结肠切除术和女性患儿肝移植的危险因素。
