中华内科杂志
中華內科雜誌
중화내과잡지
CHINESE JOURNAL OF INTERNAL MEDICINE
2008年
5期
362-365
,共4页
结缔组织病%弥漫性肺泡出血
結締組織病%瀰漫性肺泡齣血
결체조직병%미만성폐포출혈
Connective tissue diseases%Diffuse alveolar hemorrhage
目的 通过分析总结弥漫性结缔组织病(CTD)合并弥漫性肺泡出血(DHA)的临床资料,提高对其的认识.方法 回顾分析北京协和医院2004年4月-2007年6月确诊的32例弥漫性CTD并发DAH的临床特点和诊疗方法.结果 弥漫性CTD合并DAH的患者中,显微镜下多血管炎(MPA)发病率为58.8%(10/17),系统性红斑狼疮(SLE)为1.5%(19/1267),韦格纳肉芽肿(WG)为3.6%(2/56),类风湿关节炎(RA)为0.2%(1/570);诊断DAH时的年龄:SLE(27.3±13.1)岁,MPA(50.1±20.7)岁;诊断DAH时的病程:SLE(16.7±18.3)个月,MPA(10.6士18.7)个月;SLE合并DAH时SLE疾病活动指数(SLEDAI)评分为(17.1 4±6.7)分,抗双链DNA抗体呈高滴度,补体c3为(41.8±25.2)ms/dl.WG、MPA、RA患者ESR、C反应蛋白均明显升高,MPA/WG患者髓过氧化物酶抗体(MPO)/丝氨酸蛋白酶抗体(PR3)呈高滴度阳性;DAH主要表现是咯血、胸闷、呼吸困难、Hb下降;X线胸片、高分辨率CT可出现异常.多肺段支气管肺泡灌洗诊断DAH阳性率为100%.20例患者(20/32,62.5%)发生肺部感染,其中真菌感染10例(10/20,50%),混合性感染10例(10/20,50%).死亡19例(19/32,59.4%),其中SLE 12例(12/19,63.2%),MPA 5例(5/10),WG 2例(2/2);直接死于呼吸衰竭12例(12/19,63.2%).结论 弥漫性CTD患者出现咯血、呼吸困难,不明原因的Hb下降,胸部x线和高分辨率CT出现弥漫性浸润影,应警惕DAH,支气管肺泡灌洗有助于确诊.DAH与弥漫性CTD原发病的活动性密切相关,容易导致呼吸衰竭和肺部感染.弥漫性CTD合并DAH病死率较高,应早期诊断,早期治疗.
目的 通過分析總結瀰漫性結締組織病(CTD)閤併瀰漫性肺泡齣血(DHA)的臨床資料,提高對其的認識.方法 迴顧分析北京協和醫院2004年4月-2007年6月確診的32例瀰漫性CTD併髮DAH的臨床特點和診療方法.結果 瀰漫性CTD閤併DAH的患者中,顯微鏡下多血管炎(MPA)髮病率為58.8%(10/17),繫統性紅斑狼瘡(SLE)為1.5%(19/1267),韋格納肉芽腫(WG)為3.6%(2/56),類風濕關節炎(RA)為0.2%(1/570);診斷DAH時的年齡:SLE(27.3±13.1)歲,MPA(50.1±20.7)歲;診斷DAH時的病程:SLE(16.7±18.3)箇月,MPA(10.6士18.7)箇月;SLE閤併DAH時SLE疾病活動指數(SLEDAI)評分為(17.1 4±6.7)分,抗雙鏈DNA抗體呈高滴度,補體c3為(41.8±25.2)ms/dl.WG、MPA、RA患者ESR、C反應蛋白均明顯升高,MPA/WG患者髓過氧化物酶抗體(MPO)/絲氨痠蛋白酶抗體(PR3)呈高滴度暘性;DAH主要錶現是咯血、胸悶、呼吸睏難、Hb下降;X線胸片、高分辨率CT可齣現異常.多肺段支氣管肺泡灌洗診斷DAH暘性率為100%.20例患者(20/32,62.5%)髮生肺部感染,其中真菌感染10例(10/20,50%),混閤性感染10例(10/20,50%).死亡19例(19/32,59.4%),其中SLE 12例(12/19,63.2%),MPA 5例(5/10),WG 2例(2/2);直接死于呼吸衰竭12例(12/19,63.2%).結論 瀰漫性CTD患者齣現咯血、呼吸睏難,不明原因的Hb下降,胸部x線和高分辨率CT齣現瀰漫性浸潤影,應警惕DAH,支氣管肺泡灌洗有助于確診.DAH與瀰漫性CTD原髮病的活動性密切相關,容易導緻呼吸衰竭和肺部感染.瀰漫性CTD閤併DAH病死率較高,應早期診斷,早期治療.
목적 통과분석총결미만성결체조직병(CTD)합병미만성폐포출혈(DHA)적림상자료,제고대기적인식.방법 회고분석북경협화의원2004년4월-2007년6월학진적32례미만성CTD병발DAH적림상특점화진료방법.결과 미만성CTD합병DAH적환자중,현미경하다혈관염(MPA)발병솔위58.8%(10/17),계통성홍반랑창(SLE)위1.5%(19/1267),위격납육아종(WG)위3.6%(2/56),류풍습관절염(RA)위0.2%(1/570);진단DAH시적년령:SLE(27.3±13.1)세,MPA(50.1±20.7)세;진단DAH시적병정:SLE(16.7±18.3)개월,MPA(10.6사18.7)개월;SLE합병DAH시SLE질병활동지수(SLEDAI)평분위(17.1 4±6.7)분,항쌍련DNA항체정고적도,보체c3위(41.8±25.2)ms/dl.WG、MPA、RA환자ESR、C반응단백균명현승고,MPA/WG환자수과양화물매항체(MPO)/사안산단백매항체(PR3)정고적도양성;DAH주요표현시각혈、흉민、호흡곤난、Hb하강;X선흉편、고분변솔CT가출현이상.다폐단지기관폐포관세진단DAH양성솔위100%.20례환자(20/32,62.5%)발생폐부감염,기중진균감염10례(10/20,50%),혼합성감염10례(10/20,50%).사망19례(19/32,59.4%),기중SLE 12례(12/19,63.2%),MPA 5례(5/10),WG 2례(2/2);직접사우호흡쇠갈12례(12/19,63.2%).결론 미만성CTD환자출현각혈、호흡곤난,불명원인적Hb하강,흉부x선화고분변솔CT출현미만성침윤영,응경척DAH,지기관폐포관세유조우학진.DAH여미만성CTD원발병적활동성밀절상관,용역도치호흡쇠갈화폐부감염.미만성CTD합병DAH병사솔교고,응조기진단,조기치료.
Objective To provide clues to diagnosis and treatment for diffuse alveolar hemorrhage (DAH)in patients with diffuse connective tissue diseases(CTD).Method To analyze restropeetively the data of clinical features,pulmonary images and bronchoalveolar lavage(BAL)in 32 patients hospitalized in Peking Union Medical College Hospital from April 2004 to June 2007.Results The data from 1O of the 17 (58.8%)patients with microscopic polyangiitis(MPA),19 of the 1267(1.5%)patients with systemic lupus erythematosus(SLE),2 of the 56(3.6%)patients with Wegener's granulomatosis(WG)and 1 of the 570(0.2%)patients with rheumatoid arthritis(RA)were consistent with the diagnosis of DAH.DAH in SLE occurred in younger patients(mean age at the time of diagnosis 27.3±13.1 years)and early in the course of disease(median duration of SLE from onset was 16.7±18.3 months),while these figures in MPA patients with DAH were 50.1±20.7 years and 10.6±18.7 months.At the time of DAH in SLE.the median systemic lupus erythematosus disease activity index(SLEDAI)score was 17.1±6.7 and anti-ds-DNA antibody titer elevated markedly,while the median C3 level was low.The levels of erythrocyte sedimentation rate(ESR)and C-reactive protein(CRP)of patients with DAH in MPA,WG and RA showed marked elevation.The titer of antieutrophil cytoplasmic antibody(ANCA,MPO/PR3)in patients with MPA and WG was highly positive.The main clinical manifestations of DAH were hemoptysis,dyspnea and rapid decrease of hemoglobulin and hematoerit(HCT)in peripheral blood.Most patients presented with difluse alveolar infiltration on chest X-ray and hish resolution CT.DAH could be confirmed by bloody bronchoscopic lavage.20 patients(62.5%)had secondary pulmonary infections at the time of DAH;fungus and combined bacterial infection were most frequently seen.The mortalitv 0f CTD with DAH was 59.4%(19 out of 32).12 patients (63.2%)with SLE,5 patients(50%)with MPA and both of the 2 patients with WG died.12 of the lethal cases(63.2%)died of respiratory failure.Conclusions CTD patients presenting with hemoptysis and dyspnea with rapid decrease of hemoglobulin,and diffuse alveolar infiltration on chest X-ray or high resolution CT should be seriously considered to be suffering from DAH.A bloody BAL may confirm the diagnosis of DAH.DAH in CTD is an acute,serious and frequently life-threatening situation resulting in respiratory hilure and pulmonary infection.It is important for CTD patients with DAH to be diagnosed early and treated vigorously.
