风湿病合并消化道穿孔患儿的临床特点分析

Clinical characteristics of ten children with rheumatic diseases complicated with digestive tract perforation

目的总结风湿病(CRDs)合并消化道(DT)穿孔患儿的临床特点、治疗及预后。方法回顾性分析2013年1月至2021年9月首都儿科研究所附属儿童医院收治的10例合并DT穿孔的CRDs患儿的临床表现、实验室检查、影像学特点、诊治及转归。结果男女比例为1∶1,其中幼年皮肌炎(JDM)7例,儿童白塞综合征(BS)2例,全身型幼年特发性关节炎(SJIA)1例,分别占同期住院患儿的2.3%(7/302)、1.2%(2/165)和0.4%(1/286)。起病年龄8个月至9.5岁,平均(5.2±2.7)岁。7例JDM患儿抗核基质蛋白2抗体(NXP2)均强阳性。6例JDM患儿儿童肌炎评定量表评分(CMAS)<5分。7例JDM和1例BS患儿均接受糖皮质激素及多种免疫抑制剂治疗,但原发病持续不缓解;1例BS和1例SJIA患儿尚未接受正规糖皮质激素及免疫抑制剂治疗。中位DT穿孔时间为原发病确诊后的9个月(1~34个月)。临床表现均有不能缓解的腹痛,部分患儿伴发热。10例均存在腹膜炎,6例诊断为脓毒症,其中4例出现脓毒症休克,4例存在肺部感染,2例造瘘口感染,3例明确腹膜后脓肿或盆腔脓肿。穿孔后9例均接受大剂量甲泼尼龙冲击治疗,8例接受环磷酰胺冲击治疗,4例(例2、6、8、9)同时联合2种或3种免疫抑制剂或生物制剂。7例行DT穿孔修补或造瘘术。1例(例3)因经济原因放弃治疗。随访时间为6~48个月,4例存活,6例死亡。结论CRDs治疗效果不佳,若出现持续腹痛,应该警惕DT穿孔,特别是NXP2阳性的JDM。该病死亡率高,积极治疗原发病是关键,同时控制感染、外科对症、营养支持。

Objective To summarize the clinical features,treatment,and prognosis of ten children with childhood rheumatic diseases(CRDs)complicated with digestive tract(DT)perforation.Methods The clinical manifestations,laboratory tests,imaging characteristics,diagnosis,treatment,and prognosis of ten children with CRDs complicated with DT perforation admitted to the Children's Hospital of Capital Institute of Pediatrics from January 2013 to September 2021 were retrospectively analyzed.Results The male-to-female ratio was 1∶1.There were seven cases of juvenile dermatomyositis(JDM),two cases of Behcet syndrome(BS),and one case of systemic juvenile idiopathic arthritis(SJIA),accounting for 2.3%(7/302),1.2%(2/165),and 0.4%(1/286)of the hospitalized children during the same period,respectively.The mean age of onset was(5.2±2.7)years(range,8 months to 9.5 years).Seven children with JDM were strongly positive for antinuclear matrix protein 2 antibody(NXP2).The Childhood Myositis Assessment Scale(CMAS)score of six children with JDM was<5 points.Seven children with JDM and one child with BS received glucocorticoids and various immunosuppressants,but the primary disease continued to be unresolved.One child with BS and one with SJIA did not receive regular glucocorticoids and immunosuppressants.The median DT perforation time was 9 months(range,1-34 months)after the diagnosis of the primary disease.The main clinical manifestation was unrelieved abdominal pain,and some children were accompanied by fever.All ten cases had peritonitis.After perforation,nine patients received high-dose methylprednisolone pulse therapy,eight received cyclophosphamide pulse therapy,and four(cases 2,6,8,and 9)received combined therapy with two or three immunosuppressants or biological agents.Seven children underwent routine DT perforation repair or ostomy.One patient(case 3)gave up treatment for economic reasons.Four cases survived,and the follow-up time was 6-48 months;six cases died.Conclusions The treatment efficacy of CRDs is unsatisfactory,and if persistent abdominal pain occurs,DT perforation should be suspected,especially in cases with NXP2-positive JDM.The mortality rate is high,and active treatment of the primary disease is the key,along with infection control,symptomatic surgical therapy,and nutritional support.