Paediatric Hepatology, Gastroenterology and Transplantation, Hospital Papa Giovanni XXIII, Piazza Oms 1, 24127, Bergamo, Italy.
Liver Pathology, Hospital Papa Giovanni XXIII, Bergamo, Italy.
Hepatol Int. 2019 Jul;13(4):510-518. doi: 10.1007/s12072-019-09948-1. Epub 2019 May 8.
Various degrees of biliary changes are considered to be part of the histological picture of children with pediatrics autoimmune liver disease (AILD), but the literature is scarce and confusing. We aimed to describe the characteristics of children with AILD (autoimmune hepatitis, AIH, and autoimmune sclerosing cholangitis, ASC) focusing on the prevalence and type of biliary abnormalities on initial biopsy to see whether ASC was predictable on histological ground.
The files of children diagnosed with AILD were reviewed. The Ishak score was used to grade inflammation and fibrosis on biopsy; a biliary score was built to grade bile duct injury. Demographic, laboratory and histological features at diagnosis were reported and compared between the two groups (AIH vs ASC).
Forty-one patients were diagnosed with AIH (n = 24), ASC (n = 13) and PSC (n = 4) between 2009 and 2018. Twenty-nine patients [F = 76%, AIH = 20, ASC = 9, median age at diagnosis 11.7 (range 2.2-17.8)] were included in the study; 12 (4 with PSC) were excluded. Prevalence of inflammatory bowel disease was higher in ASC group (56% vs 10% in AIH, p < 0.05). On histology 17% had cirrhosis. The grade of biliopathy with AILD was moderate in 72% and severe in 31%, and overall more prominent in ASC (p = 0.031). The inflammation of the bile ducts was classified as "multifocal" or "diffuse" mainly in ASC patients (89% vs 45% in AIH, p = 0.043). Periductular fibrosis was reported in 52% of AILD patients, with a higher mean score in ASC group (p < 0.05). However, ductular reaction, biliary metaplasia and granulomatous cholangitis were equally reported in AIH and ASC, providing no clear-cut for the distinction of the two entities in the global histological evaluation.
Majority of patients with pediatrics AILD have "moderate" or "severe" features of biliopathy; AIH and ASC are not easily distinguishable on histological ground at diagnosis, and therefore, the cholangiogram remains the only effective tool to differentiate patients with AIH from those with ASC. Further prospective studies are needed to better define histological biliary features in AILD, assess if the biliopathy responds to immunosuppressive treatment and evaluate its impact on long-term outcome.
各种程度的胆道变化被认为是儿科自身免疫性肝病（AILD）组织学表现的一部分，但文献稀少且存在混淆。我们旨在描述 AILD 患儿的特征（自身免疫性肝炎、AIH 和自身免疫性硬化性胆管炎、ASC），重点描述初始活检时胆道异常的发生率和类型，以观察 ASC 是否可以从组织学上预测。
回顾诊断为 AILD 的患儿的病历。采用 Ishak 评分对活检时的炎症和纤维化进行分级；建立胆道评分以分级胆管损伤。报告诊断时的人口统计学、实验室和组织学特征，并比较两组（AIH 与 ASC）之间的特征。
2009 年至 2018 年间，诊断为 AIH（n=24）、ASC（n=13）和 PSC（n=4）的 41 例患儿。29 例患儿[F=76%，AIH=20 例，ASC=9 例，诊断时的中位年龄为 11.7（2.2-17.8）岁]被纳入研究；12 例（包括 4 例 PSC 患儿）被排除。ASC 组炎症性肠病的患病率更高（56% vs AIH 组的 10%，p<0.05）。组织学上 17%的患儿存在肝硬化。AIH 患儿的胆道病变分级为中度（72%）和重度（31%），ASC 患儿的胆道病变分级更显著（p=0.031）。胆管炎的炎症主要表现为“多灶性”或“弥漫性”，主要见于 ASC 患儿（89% vs AIH 组的 45%，p=0.043）。52%的 AILD 患儿存在胆管周围纤维化，ASC 组的平均评分较高（p<0.05）。然而，胆管反应、胆管化生和肉芽肿性胆管炎在 AIH 和 ASC 中同样有报道，因此在整体组织学评估中，这两种疾病难以区分。
大多数儿科 AILD 患儿存在“中度”或“重度”胆道病变；AIH 和 ASC 在组织学上难以区分，因此胆管造影仍然是区分 AIH 患儿与 ASC 患儿的唯一有效方法。需要进一步的前瞻性研究来更好地定义 AILD 的组织学胆道特征，评估其对免疫抑制治疗的反应，并评估其对长期预后的影响。
