Little Rebecca, Putra Juan, Kamath Binita M, Griffiths Anne M, Ricciuto Amanda, Siddiqui Iram
Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, Ontario, Canada.
Division of Pathology, The Hospital for Sick Children, Toronto, Ontario, Canada.
J Pediatr Gastroenterol Nutr. 2025 Feb;80(2):290-299. doi: 10.1002/jpn3.12434. Epub 2024 Dec 17.
We aimed to characterize the histologic gut phenotype of pediatric primary sclerosing cholangitis (PSC)-associated inflammatory bowel disease (IBD) against non-PSC colitis, and to assess Nancy Index (NI) performance in pediatric PSC-IBD.
Single-center retrospective cohort study including children diagnosed with PSC-IBD or non-PSC colitis (ulcerative colitis [UC] or IBD-unclassified) from 2000 to 2018, with diagnostic intestinal biopsies. Biopsies were re-reviewed by two independent pathologists who assessed microscopic disease distribution, NI scores, and specific histological features in the right and left colons, overall and stratified by endoscopic severity (moderate-severe vs. no more than mild). We examined NI inter-rater reliability with Fleiss' weighted (quadratic) kappa and NI construct validity against global endoscopic severity (Spearman correlation) and clinical outcomes (logistic regression).
Fifty children with PSC-IBD and 81 colitis controls were included. Histologically, pancolitis (84% vs. 55%), right colon-predominant colitis (48% vs. 3%), and backwash ileitis (53% vs. 12%) (all p < 0.01) were significantly more common in PSC-IBD; histologic rectal sparing occurred at similar rates (6% vs. 10%, p = 0.54). Lamina propria-predominant neutrophils, prominent eosinophilic infiltration (left colon), and surface villiform change (right colon) were more common in PSC-IBD than colitis controls (p < 0.01). NI showed excellent inter-rater reliability (kappa > 0.9) and correlated moderately with global endoscopic severity but poorly with clinical activity in PSC-IBD.
Pediatric PSC-IBD has a distinct histologic phenotype that largely mirrors the endoscopic phenotype in distribution and includes a greater frequency of features not included in conventional UC histologic activity indices. Future work should investigate whether a PSC-IBD-specific index incorporating these features is warranted.
我们旨在描述儿童原发性硬化性胆管炎（PSC）相关炎症性肠病（IBD）相对于非PSC结肠炎的肠道组织学表型，并评估南希指数（NI）在儿童PSC-IBD中的表现。
单中心回顾性队列研究，纳入2000年至2018年诊断为PSC-IBD或非PSC结肠炎（溃疡性结肠炎[UC]或未分类的IBD）的儿童，并进行诊断性肠道活检。活检标本由两名独立病理学家重新评估，他们评估显微镜下疾病分布、NI评分以及左右结肠的特定组织学特征，整体评估并按内镜严重程度分层（中度-重度与不超过轻度）。我们使用Fleiss加权（二次）kappa检验NI评分者间信度，并通过Spearman相关性分析NI与整体内镜严重程度以及逻辑回归分析NI与临床结局的结构效度。
纳入50例PSC-IBD患儿和81例结肠炎对照。组织学上，全结肠炎（84%对55%）、以右半结肠为主的结肠炎（48%对3%）和反流性回肠炎（53%对12%）（均p<0.01）在PSC-IBD中显著更常见；组织学上直肠 spared发生率相似（6%对10%，p = 0.54）。固有层为主的中性粒细胞、显著的嗜酸性粒细胞浸润（左半结肠）和表面绒毛状改变（右半结肠）在PSC-IBD中比结肠炎对照更常见（p<0.01）。NI显示出极好的评分者间信度（kappa>0.9），与整体内镜严重程度中度相关，但与PSC-IBD的临床活动相关性较差。
儿童PSC-IBD具有独特的组织学表型，在分布上很大程度上反映内镜表型，并且包括传统UC组织学活动指数未涵盖的更高频率特征。未来工作应研究是否需要纳入这些特征的PSC-IBD特异性指数。
