Objectives: Henoch Schönlein Purpura (HSP) is the most common systemic vasculitis in childhood. We aimed to evaluate the clinical features, seasonal variation, treatment outcomes and the possible predicting factors related to outcome among a large cohort of pediatric HSP patients.Methods: We conducted a medical record review study between July 2016 and January 2019 and evaluated the clinical manifestations and potential risk factors for severe gastrointestinal (GI) involvement, biopsy-proven nephritis and relapses.Results: The study included 420 HSP patients, of which the mean age at diagnosis was 7.68 ± 3.15 years. Clinical manifestations were arthralgia and/or arthritis (n = 244, 58.1%), abdominal pain (n = 235, 56%), subcutaneous edema (n = 163, 38.8%), and renal involvement (n = 125, 29.8%). Disease recurred for at least once, in 69 (16.4%) patients and colchicine treatment yielded a favorable response in 11 of 12 relapsing patients, who did not respond to ibuprofen or steroids. Frequencies of renal involvement and biopsy-proven nephritis were higher in patients with severe GI involvement. Besides, patients with biopsy-proven nephritis had higher rates of abdominal pain, intussusception, severe GI involvement, and systemic steroid administration.Conclusion: We speculate that renal involvement, biopsy-proven nephritis and severe GI involvement can be related to each other. Colchicine may be effective in patients with relapsing disease.

译文

目的: 过敏性紫癜 (HSP) 是儿童时期最常见的系统性血管炎。我们旨在评估一个大型队列的小儿HSP患者的临床特征,季节变化,治疗结果以及与结果相关的可能预测因素。方法: 我们在2016年7月和2019年1月之间进行了病历回顾研究,并评估了严重胃肠道 (GI) 受累的临床表现和潜在危险因素。结果: 本研究纳入420例HSP患者,诊断时平均年龄7.68   ±   3.15岁。临床表现为关节痛和/或关节炎 (n   =   244,58.1%),腹痛 (n   =   235,56%),皮下水肿 (n   =   163,38.8%) 和肾脏受累 (n   =   125,29.8%)。在69名 (16.4% 名) 患者中,疾病至少复发一次,秋水仙碱治疗在对布洛芬或类固醇无反应的12名复发患者中,有11名获得了良好的反应。严重GI受累的患者中,肾脏受累和经活检证实的肾炎的频率更高。此外,经活检证实的肾炎患者的腹痛,肠套叠,严重的GI受累和全身类固醇给药的发生率更高。结论: 我们推测肾脏受累,经活检证实的肾炎和严重的GI受累可能相互相关。秋水仙碱可能对复发性疾病患者有效。

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