Association between serum IgG level and clinical course in primary sclerosing cholangitis
Association between serum IgG level and clinical course in primary sclerosing cholangitis
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DOI：
10.1186/s12876-019-1075-0
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发表时间：
2019-08-27
影响因子：
2.4
通讯作者：
Rupp, Christian
中科院分区：
文献类型：
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作者：
Hippchen, Theresa;Sauer, Peter;Rupp, Christian
背景：原发性硬化性胆管炎是一种慢性胆汁淤积性肝病。其发病机制尚不完全清楚，但有证据表明免疫介导的过程可能有助于疾病进展。方法：在一组明确的PSC患者队列中，我们研究了血清免疫球蛋白G （IgG）在初始诊断时高于正常上限作为免疫激活标志物与预后的相关性及其对无移植生存的影响。结果：最终研究队列包括148名PSC患者。66例（44.6%）患者IgG水平升高。除了首次诊断时年龄较年轻外，IgG水平升高或不升高的患者之间没有显著差异。同时存在炎症性肠病、自身免疫性肝炎或免疫抑制药物的患者在两组中分布均匀。IgG水平升高的患者达到联合终点(34例（59.6%）vs. 23例（40.4%）；p = 0.004)，且无移植生存期降低(Log-rank: 24.0 (10.2-37.9) vs. 14.0 (8.5-19.5)；P < 0.05)。Cox回归分析包括年龄、性别、是否存在IBD、是否存在显性狭窄（DS）、Mayo Risk Score （MRS）、免疫抑制、UDCA生化反应和IgG水平升高证实MRS （p = 0.03）、DS （p = 0.04）、生化反应（p = 0.04）和IgG水平升高（p = 0.04）为降低无移植生存期的独立危险因素。结论：我们发现首次诊断时血清IgG水平升高是PSC患者无移植生存降低的独立危险因素。
Background: Primary sclerosing cholangitis is a chronic cholestatic liver disease. The pathomechanism is still not fully understood, but there is evidence that immune-mediated processes may contribute to disease progression.Methods: We studied the prognostic relevance of serum immunoglobulin G (IgG) elevated above the upper limit of normal as a marker for immune activation at initial diagnosis and its influence on transplantation-free survival in a well-defined cohort of PSC patients.Results: The final study cohort comprises of 148 PSC patients. Elevated IgG levels were found in 66 patients (44.6%). Apart from their younger age at first diagnosis, there was no significant difference between patients with or without elevated IgG levels. The presence of a concomitant inflammatory bowel disease, an autoimmune hepatitis or immunosuppressive medication was equally distributed between both groups. Patients with elevated IgG levels reached the combined endpoint (34 (59.6%) vs. 23 (40.4%); p = 0.004) significantly more often and had reduced transplantation-free survival (Log-rank: 24.0 (10.2-37.9) vs. 14.0 (8.5-19.5); p < 0.05). Cox regression analysis including age, gender, presence of IBD, presence of dominant stricture (DS), Mayo Risk Score (MRS), immunosuppression, biochemical response to UDCA and elevated IgG-levels confirmed MRS (p = 0.03), DS (p = 0.04), biochemical response (p = 0.04) and elevated IgG level (p = 0.04) as independent risk factors for reduced transplantation-free survival.Conclusion: We identified elevated serum IgG levels at first diagnosis as an independent risk factor for reduced transplant free-survival in patients with PSC.