Department of Internal Medicine, Peking Union Medical College Hospital, Beijing 100730, China.
J Clin Rheumatol. 2009 Oct;15(7):341-4. doi: 10.1097/RHU.0b013e3181b59581.
To summarize the clinical features and therapeutic response of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis complicated with diffuse alveolar hemorrhage (DAH).
A retrospective chart review of the patients having ANCA-associated vasculitis with DAH was made with regard to their clinical symptoms, laboratory test results, responses to therapy and outcomes.
During January 1994 to June 2007, 131 ANCA-associated vasculitis patients were admitted to Peking Union Medical College Hospital. During this period, 12 of these cases consisting of 9 males and 3 females with a mean age of 59.9 +/- 16.7 years developed DAH. The mean duration of vasculitis before the onset of DAH was 9.0 +/- 14.3 months (range: 0-48 months). Dyspnea was the most consistent presenting symptom, while frank hemoptysis occurred in only 5 cases at the onset of DAH. The most common extrapulmonary findings associated with DAH were glomerulonephritis (100%). Symptoms including fever and arthralgia-myalgia (91.7%) as well as complications involving gastrointestinal (41.7%), mucocutaneous (25%), ear-nose-throat (25%), cardiovascular (16.7%), and nervous system (16.7%) were also frequently seen. The Birmingham Vasculitis Activity Score at the onset of DAH was 21.8 +/- 4.9. All patients were treated with systemic corticosteroids combined with cyclophosphamide including methylprednisolone pulse therapy in 7 patients. In addition, plasmapheresis (41.7%), dialysis (25%), and mechanical ventilation (41.7%) were applied. The overall mortality rate was 58.3% (7 patients). Three patients died of fulminant DAH and respiratory failure during the first week of treatment. The other 4 patients died of septic shock (2 patients), severe heart failure (1 patient), and systemic fungus infection with septic shock (1 patient) 10 to 32 days after the onset of DAH.
DAH is the most serious complication of ANCA-associated vasculitis. The episode of DAH always occurs simultaneously with multiple system involvement. The most constant signs are newly developed dyspnea and new infiltration of bilateral lungs. Prompt bronchoalveolar lavage can be helpful for timely diagnosis of the patients without overt hemoptysis and a useful tool to exclude pulmonary infection.
总结抗中性粒细胞胞浆抗体（ANCA）相关性血管炎合并弥漫性肺泡出血（DAH）患者的临床特征和治疗反应。
对 1994 年 1 月至 2007 年 6 月期间因 DAH 住院的 ANCA 相关性血管炎患者的临床症状、实验室检查结果、治疗反应和结局进行回顾性图表分析。
在这期间，131 例 ANCA 相关性血管炎患者中，12 例（9 例男性，3 例女性）出现 DAH，平均年龄 59.9±16.7 岁。在 DAH 发作前，血管炎的平均持续时间为 9.0±14.3 个月（范围：0-48 个月）。呼吸困难是最常见的首发症状，而在 DAH 发作时仅 5 例出现明显咯血。与 DAH 相关的最常见的肺外表现是肾小球肾炎（100%）。伴有发热和关节痛-肌痛（91.7%）的症状以及累及胃肠道（41.7%）、黏膜皮肤（25%）、耳鼻喉（25%）、心血管（16.7%）和神经系统（16.7%）的并发症也很常见。在 DAH 发作时，Birmingham 血管炎活动评分（Birmingham Vasculitis Activity Score，BVAS）为 21.8±4.9。所有患者均接受全身皮质类固醇激素联合环磷酰胺治疗，其中 7 例接受甲泼尼龙脉冲治疗。此外，还应用了血浆置换（41.7%）、透析（25%）和机械通气（41.7%）。总死亡率为 58.3%（7 例）。3 例患者在治疗的第一周死于暴发性 DAH 和呼吸衰竭。另外 4 例患者分别在 DAH 发作后 10-32 天死于感染性休克（2 例）、严重心力衰竭（1 例）和系统性真菌感染合并感染性休克（1 例）。
DAH 是 ANCA 相关性血管炎最严重的并发症。DAH 发作总是同时伴有多系统受累。最常见的体征是新出现的呼吸困难和双侧肺部新浸润。及时进行支气管肺泡灌洗有助于对没有明显咯血的患者进行及时诊断，也是排除肺部感染的有用工具。
