Clinical analysis of 4 cases of juvenile dermatomyositis complicated with massive hemorrhage of digestive tract
10.3760/cma.j.cn112140-20250312-00201
- VernacularTitle:幼年皮肌炎合并消化道大出血4例临床分析
- Author:
Xinning WANG
1
;
Xiaolei WANG
1
;
Jinru ZHANG
1
;
Xin YAO
1
;
Yuan LIU
1
;
Jianguo LI
1
Author Information
1. 首都医科大学附属首都儿童医学中心风湿免疫科，北京 100020
- Publication Type:Journal Article
- Keywords:
Dermatomyositis;
Child;
Anti-NXP2 antibody;
Sever gastrointestinal hemorrhage
- From:
Chinese Journal of Pediatrics
2025;63(7):789-793
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical characteristics, treatment and prognosis of juvenile dermatomyositis (JDM) complicated by sever gastrointestinal hemorrhage in children.Methods:A retrospective analysis was conducted on 4 JDM patients with sever gastrointestinal hemorrhage admitted to our hospital, from January 2017 to January 2025. Data including demographics, clinical manifestations, laboratory and imaging findings, treatment courses, and outcomes were reviewed.Results:The cohort comprised 4 patients (2 males, 2 females), with onset ages of 6.1, 6.2, 10.0 and 8.0 years. All presented with rash and fatigue and were diagnosed with severe refractory JDM (strongly positive anti-NXP2 antibodies). Sever gastrointestinal hemorrhage occurred 18, 12, 51, and 2 months after JDM diagnosis. Two cases had confirmed gastrointestinal infections due to contaminated food. Abdominal pain was the initial gastrointestinal symptom and black stool was observed in all cases, hemoglobin levels dropped below 60 g/L (case 1-4 decreased to 38, 59, 60 and 43 g/L respectively). All patients exhibited intestinal wall thickening. Active bleeding sites included the duodenum (3 cases: 2 cases near the duodenal papilla, 1 cases with diffuse duodenal oozing). Emergency endoscopic hemostasis was performed in 3 cases. One patient with diffuse duodenal bleeding required additional glucocortieoid pulse therapy after failed interventional embolization. Three patients stabilized following aggressive treatment of JDM, while 1 case died due to duodenal perforation.Conclusions:Anti-NXP2 antibody-positive JDM patients are prone to gastrointestinal involvement, particularly in chronic cases. Duodenal bleeding is common, with vascular erosion and deep mucosal ulcers posing life-threatening risks. For children with positive anti-NXP2 antibodies who present with abdominal pain and thickened intestinal walls, early endoscopic examination should be conducted as soon as possible to detect digestive tract lesions and provide timely treatment. Patients with severe digestive tract bleeding usually require active treatment for the underlying disease combined with endoscopic hemostasis therapy. Under timely treatment, the prognosis is relatively favorable.
