Department of Emergency Medicine, Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Taoyuan, Taiwan.
Semin Arthritis Rheum. 2011 Oct;41(2):305-12. doi: 10.1016/j.semarthrit.2011.03.007.
To characterize the clinical characteristics of pediatric patients with pulmonary hemorrhage and Henoch-Schönlein purpura (HSP).
Presentation of a pediatric patient with pulmonary hemorrhage associated with HSP and review of relevant cases based on a PubMed search (1966 to April 2010).
We identified 17 previously reported pediatric cases with HSP and pulmonary hemorrhage. The most frequent clinical manifestations were rash (17 patients, 100%), proteinuria (14 patients, 82%), and abdominal pain (13 patients, 76%). Six patients (35%) had complete resolution of symptoms; 7 patients (41%) had partial recovery, and 4 patients (23%) died. Nine patients (53%) had acute respiratory failure following intubation and 3 of these patients (33%) died. Five patients were treated with methylprednisolone pulse therapy and 1 was also given low-dose cyclophosphamide treatment, but 2 of these 5 patients (40%) died. Three patients were given cyclophosphamide pulse therapy plus steroid (nonpulse or pulse) therapy, and all survived. Among the 6 nonintubated patients, all were given steroid treatment with or without an immunosuppressant drug, and all survived. In our reported case, plasma exchange appeared to help resolve the pulmonary hemorrhage and crescentic glomerulonephritis that were associated with HSP.
For pediatric HSP patients with pulmonary hemorrhage but no respiratory failure, methylprednisolone pulse or nonpulse therapy could be the first-line therapy. In the presence of respiratory failure, cyclophosphamide pulse therapy is suggested. Plasma exchange may be considered for treatment of pulmonary renal syndrome or refractory pulmonary hemorrhage.
描述伴有过敏性紫癜（HSP）的小儿肺出血的临床特征。
报告一例伴有 HSP 的小儿肺出血患者，并根据 PubMed 检索（1966 年至 2010 年 4 月）对相关病例进行综述。
共确定了 17 例以前报道的 HSP 合并肺出血的儿科病例。最常见的临床表现为皮疹（17 例，100%）、蛋白尿（14 例，82%）和腹痛（13 例，76%）。6 例（35%）患者症状完全缓解；7 例（41%）患者部分恢复，4 例（23%）患者死亡。9 例（53%）患者在插管后出现急性呼吸衰竭，其中 3 例（33%）死亡。5 例患者接受甲基强的松龙冲击治疗，1 例患者接受小剂量环磷酰胺治疗，但其中 2 例（40%）死亡。3 例患者接受环磷酰胺冲击治疗加类固醇（非冲击或冲击）治疗，均存活。在 6 例非插管患者中，所有患者均接受了类固醇治疗加或不加免疫抑制剂药物治疗，均存活。在我们报告的病例中，血浆置换似乎有助于缓解 HSP 相关的肺出血和新月体肾炎。
对于伴有肺出血但无呼吸衰竭的小儿 HSP 患者，甲基强的松龙冲击或非冲击治疗可作为一线治疗。存在呼吸衰竭时，建议使用环磷酰胺冲击治疗。对于肺-肾综合征或难治性肺出血，可考虑采用血浆置换治疗。
