Department of Pulmonology, St John's Medical College Hospital, Sarjapur Road, Bangalore, India.
Semin Arthritis Rheum. 2013 Feb;42(4):391-400. doi: 10.1016/j.semarthrit.2012.07.004. Epub 2012 Sep 15.
Diffuse alveolar hemorrhage (DAH) is a rare complication of Henoch-Schönlein purpura (HSP) and data on its prevalence, management, and outcomes are scant.
To enable evidence-based management of DAH in HSP.
A case report and a systematic review were conducted of all reported cases of DAH complicating HSP in the English literature.
DAH predominantly affects older male children and adults with HSP. The occurrence of DAH in HSP is rare and the reported prevalence ranged from 0.8% to 5%. DAH occurred variably after the diagnosis of HSP, ranging from 2 days to 18 years. Hemoptysis (75%), drop in hemoglobin (74%), and chest infiltrates (94%) were the most common clinical findings. Lung biopsy showed leukocytoclastic vasculitis with alveolar hemorrhage (69.2%) or only alveolar hemorrhage (31.8%) with variable IgA staining by immunofluorescence. DAH was frequently severe and 50% of the patients required mechanical ventilation. Cyclophosphamide and pulse methylprednisolone for DAH was associated with better outcomes, particularly in patients who were already receiving steroids at the time of DAH. Steroids and immunosuppressants were administered for a median duration of 9 and 4.5 months, respectively. Systemic recurrences (27.7%) and recurrences of DAH (8.3%) were frequent. DAH was associated with high mortality (27.6%) and morbidity (persistent urinary abnormalities, 12%; chronic renal failure, 9%; complications of therapy, 27%).
DAH is a life-threatening complication in HSP. Current protocols use pulse methylprednisolone and cyclophosphamide for 6 months.
弥漫性肺泡出血（DAH）是过敏性紫癜（HSP）的罕见并发症，有关其患病率、治疗和结局的数据很少。
为 HSP 中 DAH 的循证管理提供依据。
对英文文献中所有报道的 HSP 并发 DAH 的病例进行了病例报告和系统评价。
DAH 主要影响年龄较大的 HSP 男性儿童和成人。HSP 中 DAH 的发生率较低，报告的患病率为 0.8%至 5%。DAH 在 HSP 诊断后出现的时间各不相同，范围从 2 天到 18 年。咯血（75%）、血红蛋白下降（74%）和胸部浸润（94%）是最常见的临床发现。肺活检显示白细胞碎裂性血管炎伴肺泡出血（69.2%）或仅肺泡出血（31.8%），免疫荧光染色时 IgA 染色可变。DAH 常很严重，50%的患者需要机械通气。环磷酰胺和脉冲甲基强的松龙治疗 DAH 效果较好，特别是在 DAH 发生时已接受激素治疗的患者。激素和免疫抑制剂的中位治疗时间分别为 9 个月和 4.5 个月。全身性复发（27.7%）和 DAH 复发（8.3%）很常见。DAH 与高死亡率（27.6%）和发病率（持续的尿液异常，12%；慢性肾衰竭，9%；治疗并发症，27%）相关。
DAH 是 HSP 的一种危及生命的并发症。目前的方案使用脉冲甲基强的松龙和环磷酰胺治疗 6 个月。
