Wang Li, Li Jing, Zhao Jing, Chen Hua, Zheng Wenjie, Wu Qingjun, Zhang Fengchun
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China; Email:
Zhonghua Nei Ke Za Zhi. 2015 May;54(5):416-9.
To investigate the clinical features of patients with microscopic polyangiitis (MPA) and alveolar hemorrhage (AH), so as to raise the physicians' understanding of this condition.
The clinical data of 15 MPA patients combined with AH, who were hospitalized in Peking Union Medical College Hospital from January 2008 to January 2014, were retrospectively analyzed.
These 15 subjects accounted for 18.1% of all MPA patients, including 4 males and 11 females with mean age of (59.4 ± 17.9) years old and mean disease duration of 7.5 (1-48) months. Hemoptysis or bloody sputum (14/15) and dyspnea (11/15) were predominant pulmonary manifestations. Hypoxemia appeared in 6 patients (40.0%). An average 8-46 (22.7 ± 4.4) g/L drop of hemoglobin level was seen in all 15 patients. Imaging revealed new bilateral infiltrations or opacities in 13 cases, and bloody bronchoalveolar lavage fluid were found in 6 cases. Compared with MPA patients without AH, the incidence of myalgia [2/15 vs 50.0%(34/68); P=0.009, OR=0.154] and hemoglobin level [(83.2 ± 21.0) g/L vs (102.5 ± 18.7) g/L; P=0.001] were statistically lower in MPA patients with AH. There were no statistical differences in age, sex, interstitial lung disease, renal involvement, peripheral neuropathy and the five factor score between two groups. The proportion of patients who received corticosteroid pulse treatment in MPA with AH group was significantly higher than that of MPA patients without AH [9/15 vs 19.1%(13/68); P=0.003, OR=6.346]. After treatment, 13 cases achieved remission, while 2 died.
As an important pulmonary manifestation of MPA, AH could be insidious but fatal. The treatment with steroid, cyclophosphamide and plasma exchange could improve the prognosis.
探讨显微镜下多血管炎（MPA）合并肺泡出血（AH）患者的临床特点，以提高医师对该病的认识。
回顾性分析2008年1月至2014年1月在北京协和医院住院的15例MPA合并AH患者的临床资料。
这15例患者占所有MPA患者的18.1%，其中男性4例，女性11例，平均年龄（59.4±17.9）岁，平均病程7.5（1 - 48）个月。咯血或血痰（14/15）和呼吸困难（11/15）是主要的肺部表现。6例患者（40.0%）出现低氧血症。15例患者血红蛋白水平平均下降8 - 46（22.7±4.4）g/L。影像学检查显示13例患者双侧出现新的浸润影或实变影，6例患者支气管肺泡灌洗为血性。与无AH的MPA患者相比，AH组MPA患者肌痛发生率[2/15 vs 50.0%（34/68）；P = 0.009，OR = 0.154]和血红蛋白水平[（83.2±21.0）g/L vs（102.5±18.7）g/L；P = 0.001]在统计学上更低。两组患者在年龄、性别、间质性肺疾病、肾脏受累、周围神经病变及五因素评分方面无统计学差异。AH组MPA患者接受糖皮质激素冲击治疗的比例显著高于无AH的MPA患者[9/15 vs 19.1%（13/68）；P = 0.003，OR = 6.346]。治疗后，13例缓解，2例死亡。
AH作为MPA重要的肺部表现，可能隐匿但致命。应用糖皮质激素、环磷酰胺及血浆置换治疗可改善预后。
