Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Department of Pathophysiology and Transplantation, Pediatric Highly Intensive Care Unit, Università degli Studi di Milano, 20122, Milan, Italy.
ASST NORDMILANO, Ospedale di Sesto San Giovanni, Pediatric and Neonatology Unit, Sesto San Giovanni, 20099, Milan, Italy.
Pediatr Rheumatol Online J. 2019 Nov 21;17(1):75. doi: 10.1186/s12969-019-0381-y.
Henoch-Schönlein Purpura (HSP) is the most common vasculitis of childhood and affects the small blood vessels. Pulmonary involvement is a rare complication of HSP and diffuse alveolar hemorrhage (DAH) is the most frequent clinical presentation. Little is known about the real incidence of lung involvement during HSP in the pediatric age and about its diagnosis, management and outcome.
In order to discuss the main clinical findings and the diagnosis and management of lung involvement in children with HSP, we performed a review of the literature of the last 40 years.
We identified 23 pediatric cases of HSP with lung involvement. DAH was the most frequent clinical presentation of the disease. Although it can be identified by chest x-ray (CXR), bronchoalveolar lavage (BAL) is the gold standard for diagnosis. Pulse methylprednisolone is the first-line of therapy in children with DAH. An immunosuppressive regimen consisting of cyclophosphamide or azathioprine plus corticosteroids is required when respiratory failure occurs. Four of the twenty-three patients died, while 18 children had a resolution of the pulmonary involvement.
DAH is a life-threatening complication of HSP. Prompt diagnosis and adequate treatment are essential in order to achieve the best outcome.
过敏性紫癜（HSP）是儿童最常见的血管炎，影响小血管。肺部受累是 HSP 的罕见并发症，弥漫性肺泡出血（DAH）是最常见的临床表现。关于 HSP 患儿肺部受累的真实发生率以及其诊断、管理和结局，我们知之甚少。
为了讨论 HSP 患儿肺部受累的主要临床发现以及诊断和管理，我们对过去 40 年的文献进行了回顾。
我们共确定了 23 例 HSP 合并肺部受累的儿科病例。DAH 是该病最常见的临床表现。尽管 CXR 可识别 DAH，但 BAL 是诊断的金标准。对于 DAH 患儿，脉冲甲基泼尼松龙是一线治疗药物。当发生呼吸衰竭时，需要环磷酰胺或硫唑嘌呤加皮质类固醇的免疫抑制方案。23 例患者中有 4 例死亡，18 例患儿的肺部受累得到缓解。
DAH 是 HSP 的一种危及生命的并发症。及时诊断和充分治疗对于获得最佳结局至关重要。
