目的

探讨儿童系统性红斑狼疮（SLE）相关性急性胰腺炎（AP）患儿的临床诊疗及预后。

方法

选择2018年1月至2024年12月四川大学华西第二医院收治入院的24例SLE 相关性AP（AP为首发表现为7例，SLE后并发AP为17例）患儿为研究对象。采用回顾性研究方法，收集其一般临床资料、临床症状及体征、实验室检查及影像学检查结果、SLE 疾病活动性指数（SLEDAI）、治疗和转归等相关临床资料。本研究遵循程序符合2024年新修订的《世界医学会赫尔辛基宣言》要求。

结果

本研究24例患儿的临床诊疗及预后分析结果如下。①一般临床资料：24例SLE 相关性AP患儿中，SLE中位确诊年龄为12岁，女性为22例（91.7%）；重症AP为13例（54.2%）。常见消化道临床表现中，腹痛为21例（87.5%），恶心、呕吐为17例（70.8%），腹胀为11例（45.8%），中上腹压痛为19例（79.2%）。其SLEDAI评分为16～41分，均有≥2个器官/系统受累，合并急性肾损伤（AKI）者为16例（66.7%），巨噬细胞活化综合征（MAS）为4 例（16.7%）。②辅助检查结果：24 例SLE相关性AP患儿中，血淀粉酶和（或）脂肪酶较正常参考值增高、血Ca²⁺浓度较正常参考值降低、甘油三酯浓度较正常参考值增高者分别为20例（83.3%）、22例（91.7%）和20例（95.2%，20/21），腹部CT 检查结果异常者为20 例（83.3%）。17 例患儿进行腹部超声检查，结果异常者为7 例（41.2%）。④治疗与转归：对24 例患儿均采取大剂量甲基泼尼松龙冲击治疗，加用免疫抑制剂21例，进行血浆置换治疗18例。治疗后，18例患儿（75.0%）达临床缓解后出院，6例患儿（25.0%）死亡，均为重症AP者，重症AP患儿死亡率为46.2%（6/13）。

结论

SLE 相关性AP 与非SLE 的AP患儿临床表现相似，SLE后并发AP多见于青春期女性，常发生在SLE活动期，多器官/系统受累常见。SLE相关性重症AP相对多见，死亡率较高。大剂量糖皮质激素联合免疫抑制剂、血浆置换治疗可能改善SLE相关性AP患儿预后。

Objective

To explore the clinical diagnosis，treatment and prognosis of systemic lupus erythematosus （SLE）-related acute pancreatitis（AP）in children.

Methods

Twenty-four children with SLE-related AP admitted to West China Second University Hospital，Sichuan University from January 2018 to December 2024 were selected as the research subjects.AP was the initial presentation in 7 patients，and 17 patients were complicated by AP after the diagnosis of SLE.A retrospective study method was used to collect the general clinical data，clinical symptoms and signs，laboratory tests，imaging examinations，SLE disease activity index （SLEDAI），treatment and prognosis of all children.The procedures followed in this study were in line with the requirements of the newly revised World Medical Association Declaration of Helsinki in 2024.

Results

The results of clinical diagnosis，treatment and prognosis analysis of 24 children in this study were as follows.①General clinical data：Among the 24 cases of SLE-related AP children，the median age of SLE diagnosis was 12 years old，and there were 22 females （91.7%）.Severe AP accounted for 54.2%（13/24）.Common gastrointestinal manifestations included abdominal pain in 21 cases（87.5%），nausea and vomiting in 17 cases （70.8%），abdominal distension in 11 cases （45.8%），and upper abdominal tenderness in 19 cases （79.2%）.The SLEDAI score was 16-41 points.All had≥2 organs/systems involved，with acute kidney injury（AKI）in 16 cases （66.7%）and macrophage activation syndrome（MAS）in 4 cases （16.7%）.②Results of auxiliary examinations：Among the 24 cases of SLE-related AP children，the proportions of elevated serum amylase and（or）lipase，decreased serum Ca²⁺concentration，elevated triglyceride concentration were 20 cases（83.3%），22 cases（91.7%）and 20 cases （95.2%，20/21），respectively.Abnormal abdominal CT results were found in 20 children （83.3%）.Abdominal ultrasound was performed in 17 children，and abnormalities were found in 7 children （41.2%）.④Treatment and outcome：All 24 children received high-dose methylprednisolone pulse therapy，21 children were given immunosuppressants，and 18 children received plasma exchange therapy.After treatment，18 children （75.0%）achieved clinical remission and were discharged，six children （25.0%）died，all of whom were severe AP.The mortality rate of children with severe AP was 46.2%（6/13）.

Conclusions

Children with SLE-related AP has a similar clinical manifestation to AP children without SLE and occurs mostly in adolescent females，all of whom have active SLE with multiple organ system involvement.SLE-related severe AP is relatively common and has a high mortality rate.High-dose glucocorticoid therapy combined with immunosuppression and plasma exchange may lead to a better prognosis in children with SLErelated AP.