CAJ | 학술논문

目的通过分析显微镜下多血管炎(MPA)合并肺泡出血(AH)的临床特点,提高临床医生的诊治水平.方法回顾性分析北京协和医院2008年1月-2014年1月住院的83例MPA中15例(18.1％)合并AH患者的资料.结果 (1)男性4例,女性11例,年龄(59.4±17.9)岁,起病至确诊平均7.5(1 ～48)个月.(2)呼吸系统表现:咯血/痰中带血14例;呼吸困难11例;低氧血症6例;Hb下降15例[8～46(22.7±4.4) g/L];双肺弥漫浸润影13例;6例肺泡灌洗液为血性.(3)15例MPA合并AH者肌痛2例,68例MPA未合并AH者肌痛34例(P=0.009,OR=0.154).15例MPA合并AH者Hb(83.2±21.0)g/L,68例MPA未合并AH者Hb(102.5±18.7) g/L(P =0.001).两者在年龄、性别、肺间质病变、肾脏受累、消化道受累、周围神经病、五因子评分等方面差异无统计学意义.(4)15例MPA合并AH者9例采用糖皮质激素冲击治疗,68例MPA未合并AH者13例使用糖皮质激素冲击治疗(P =0.003,OR=6.346).经治疗,13例好转,2例死亡.结论 AH是MPA重要的肺部表现,临床表现隐匿,病情危重,需通过积极控制原发病、血浆置换等治疗来改善预后.
목적 통과분석현미경하다혈관염(MPA)합병폐포출혈(AH)적림상특점,제고림상의생적진치수평.방법 회고성분석북경협화의원2008년1월-2014년1월주원적83례MPA중15례(18.1％)합병AH환자적자료.결과 (1)남성4례,녀성11례,년령(59.4±17.9)세,기병지학진평균7.5(1 ～48)개월.(2)호흡계통표현:각혈/담중대혈14례;호흡곤난11례;저양혈증6례;Hb하강15례[8～46(22.7±4.4) g/L];쌍폐미만침윤영13례;6례폐포관세액위혈성.(3)15례MPA합병AH자기통2례,68례MPA미합병AH자기통34례(P=0.009,OR=0.154).15례MPA합병AH자Hb(83.2±21.0)g/L,68례MPA미합병AH자Hb(102.5±18.7) g/L(P =0.001).량자재년령、성별、폐간질병변、신장수루、소화도수루、주위신경병、오인자평분등방면차이무통계학의의.(4)15례MPA합병AH자9례채용당피질격소충격치료,68례MPA미합병AH자13례사용당피질격소충격치료(P =0.003,OR=6.346).경치료,13례호전,2례사망.결론 AH시MPA중요적폐부표현,림상표현은닉,병정위중,수통과적겁공제원발병、혈장치환등치료래개선예후.
Objective 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.Methods 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.Results 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.Conclusion 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.