Department of Pathology and Immunology, Washington University in St. Louis, MO, USA.
Department of Pediatrics, Washington University in St. Louis, MO, USA.
Clin Res Hepatol Gastroenterol. 2024 Oct;48(8):102442. doi: 10.1016/j.clinre.2024.102442. Epub 2024 Aug 4.
Autoimmune hepatitis (AIH) patients can present with advanced fibrosis at diagnosis or may progress to the same if biochemical remission on treatment is not achieved.
We conducted a single-center retrospective analysis of 34 pediatrics and 39 adult AIH patients. Three pathologists, blinded to clinical information, reviewed the diagnostic liver biopsy (DLB) slides of AIH patients. We evaluated the impact of clinical, laboratory, and histopathologic parameters on outcomes including biochemical remission (BR).
Incidence of advanced (Ludwig stage 3 or 4) fibrosis on DLB was 45.2 %. AIH patients with advanced fibrosis had higher median Ishak score (p < 0.001) and higher IgG level (p = 0.01) at diagnosis. The incidence of BR at 6-month (31.2% vs. 88.6 %, p = 0.001) and 1-year (68.8% vs. 88.6 %, p = 0.04) post-diagnosis was significantly lower in AIH patients with advanced fibrosis. Although not statistically significant, a higher proportion of AIH patients with advanced fibrosis were on high dose of steroids (58% vs. 37.9 %, p = 0.1) at 1 year post diagnosis. Higher serum IgG level at diagnosis was associated with lower odds of achieving BR at 6-month (p = 0.004) and 1-year (p = 0.03) post-diagnosis in multivariate analysis. Pediatric age at diagnosis (p = 0.02) was associated with higher steroid dose at 1-year post-diagnosis in univariate analysis.
Findings of advanced fibrosis on DLB of AIH patients was accompanied by more pronounced necro-inflammatory activity and higher serum IgG level, which translated to lower rates of BR and higher exposure to steroids during the first year after diagnosis.
自身免疫性肝炎(AIH)患者在诊断时可能已经存在晚期纤维化,或者如果治疗后未达到生化缓解,可能会进展为晚期纤维化。
我们对 34 名儿科和 39 名成人 AIH 患者进行了单中心回顾性分析。三位病理学家在不了解临床信息的情况下对 AIH 患者的诊断性肝活检(DLB)切片进行了复查。我们评估了临床、实验室和组织病理学参数对包括生化缓解(BR)在内的结局的影响。
DLB 上晚期(Ludwig 3 或 4 期)纤维化的发生率为 45.2%。AIH 患者伴有晚期纤维化时,其 Ishak 评分中位数较高(p<0.001),且诊断时 IgG 水平较高(p=0.01)。诊断后 6 个月(31.2%比 88.6%,p=0.001)和 1 年(68.8%比 88.6%,p=0.04)时 BR 的发生率明显较低。尽管差异无统计学意义,但在诊断后 1 年,伴有晚期纤维化的 AIH 患者中,接受高剂量类固醇治疗的比例较高(58%比 37.9%,p=0.1)。多变量分析显示,诊断时较高的血清 IgG 水平与 6 个月(p=0.004)和 1 年(p=0.03)时 BR 的可能性降低相关。诊断后 1 年时,儿科年龄(p=0.02)与类固醇剂量较高相关,这一结果在单变量分析中得到证实。
在 AIH 患者的 DLB 上发现晚期纤维化时,伴随着更明显的坏死性炎症活动和更高的血清 IgG 水平,这导致 BR 的发生率较低,且在诊断后 1 年内接受类固醇治疗的可能性更高。