【摘要】：背景：彌漫性肺泡出血(DAH)是系統(tǒng)性紅斑狼瘡(SLE)肺部病變中相對(duì)罕見,而進(jìn)展迅猛、死亡率較高的臨床表型,是導(dǎo)致SLE患者死亡的重要原因之一,目前臨床尚無切實(shí)有效的診治規(guī)范。目的：分析SLE-DAH患者的臨床特點(diǎn)、危險(xiǎn)因素、預(yù)后相關(guān)因素,為臨床診治提供依據(jù)。方法：回顧北京協(xié)和醫(yī)院自2004年1月至2015年1月間診斷為SLE-DAH的住院患者,同時(shí)滿足以下標(biāo)準(zhǔn)即入組：(1)符合1997年ACR或2009年SLICC的SLE分類標(biāo)準(zhǔn)；(2)同時(shí)符合以下4條標(biāo)準(zhǔn)中的3條及以上：①肺部癥狀；②新發(fā)的肺部浸潤影；③HGB下降至少15g/L；④支氣管肺泡灌洗液呈血性或可見到含鐵血黃色細(xì)胞；并可除外嚴(yán)重凝血系統(tǒng)疾病、嚴(yán)重的急性肺水腫、嚴(yán)重的肺栓塞等。同時(shí)以208例同期住院的SLE非DAH的患者作為對(duì)照進(jìn)行病例對(duì)照研究。收集全部患者的臨床表現(xiàn)、實(shí)驗(yàn)室檢查、影像學(xué)表現(xiàn)的特征,采用SPSS 20.0采集和分析數(shù)據(jù)。連續(xù)變量比較采用獨(dú)立樣本t檢驗(yàn)(其中符合方差齊性者直接用t檢驗(yàn),不符合者用近似t檢驗(yàn),不符合正態(tài)分布者用秩和檢驗(yàn)),分類變量資料采用x2檢驗(yàn)。P值0.05被認(rèn)為有統(tǒng)計(jì)學(xué)意義。結(jié)果：(1)共納入SLE-DAH患者54例,DAH在SLE患者中患病率為0.73%。其中女性49例(91%),平均年齡為(30.8±12.4)歲,平均病程為(16.9±33.6)月,1/3患者以DAH表現(xiàn)首發(fā)。平均SLEDAI評(píng)分為(19±7.9)；(2)SLE-DAH患者最常見的癥狀是呼吸困難(91%),其余依次為低氧血癥(89%)、咳嗽(87%)發(fā)熱(43例,80%),26%患者臨床無咯血；血紅蛋白平均下降(36.1±14.3)g/L,24小時(shí)內(nèi)平均下降(13.4±10.8)g/L。最常見的肺外受累為腎臟受累(91%)；(3)54例SLE-DAH患者中53例接受了糖皮質(zhì)激素治療,37例(69%)接受了甲基潑尼松龍沖擊治療；(4) SLE-DAH患者院內(nèi)病死率為20.4%；與院內(nèi)存活組相比,死亡組患者在發(fā)病年齡、病程上存在統(tǒng)計(jì)學(xué)差異,同時(shí)合并感染、機(jī)械通氣的比例更高,或與不良預(yù)后相關(guān)； (5)與SLE-NDAH患者相比,兩組患者在病程、SLEDAI評(píng)分、院內(nèi)病死率(20% vs 7%)上存在統(tǒng)計(jì)學(xué)差異,且SLE-DAH患者出現(xiàn)皮膚黏膜病變(70.4% vs 49.0%)、血液系統(tǒng)病變(87% vs 43.8%)、血小板減少(72.2% vs 25.5%)、腎臟病變(90.7% vs 62.9%)更為多見,抗ds-DNA抗體陽性率更高(55.6%vs 42.6%),以上均存在統(tǒng)計(jì)學(xué)差異(P0.05)；(6)隨著臨床認(rèn)識(shí)的增加,SLE-DAH死亡率在下降(50% vs 10%,P0.05)結(jié)論：DAH是SLE的的嚴(yán)重并發(fā)癥,臨床表現(xiàn)可無咯血表現(xiàn),應(yīng)重視支氣管鏡檢查的診斷價(jià)值。積極的糖皮質(zhì)激素、免疫抑制劑可改善預(yù)后。發(fā)病年齡、病程合并感染、應(yīng)用機(jī)械通氣與不良預(yù)后相關(guān)。提高臨床認(rèn)識(shí),可改善SLE-DAH的預(yù)后。
[Abstract]:Background: diffuse alveolar hemorrhage (DAH) is a relatively rare pulmonary lesion in systemic lupus erythematosus (SLE), which is characterized by rapid progression and high mortality, which is one of the important causes of death in patients with SLE. At present, there is no practical and effective diagnosis and treatment standard. Objective: to analyze the clinical features, risk factors and prognostic factors of SLE-DAH patients. Methods: the hospitalized patients diagnosed with SLE-DAH from January 2004 to January 2015 in Peking Union Hospital were reviewed and the following criteria were met: (1) according to the SLE classification criteria of ACR in 1997 or SLICC in 2009; (2) at the same time, 3 or more of the following 4 criteria were conformed to one or more of the following criteria. The newly developed lung infiltrating 3HGB decreased by at least 15 g / L 4 bronchoalveolar lavage fluid (BALF) with blood or iron-containing yellow cells. And may exclude the serious coagulation system disease, the serious acute pulmonary edema, the serious pulmonary embolism and so on. A case-control study was conducted with 208 non-DAH SLE patients hospitalized at the same time. The clinical, laboratory and imaging features of all patients were collected and analyzed by SPSS 20.0. The independent sample t test was used to compare the continuous variables (where the homogeneity of variance was used directly by t test, and the non-conformance was tested by approximate t test). Rank sum test was used for those who did not conform to normal distribution). The data of classified variables were analyzed by x2 test. P value 0.05 was considered to be statistically significant. Results: (1) the prevalence of SLE-DAH in 54 patients with SLE was 0.73. The mean age was (30.8 鹵12.4) years old and the mean course of disease was (16.9 鹵33.6) months / 1 / 3 of DAH in 49 women (91%). The most common symptoms in patients with SLE-DAH were dyspnea (91%), hypoxiaemia (89%), cough (87%), fever (43 cases, 80%) and hemoglobin (36.1 鹵14.3) g / L in 24 hours (13.4 鹵10.8) g / L, respectively. The average SLEDAI score was (19 鹵7.9); (2. The most common symptoms were dyspnea (91%), followed by hypoxiaemia (89%), cough (87%), fever (43 cases 80%) and no hemoptysis (26%). The most common extrapulmonary involvement was kidney involvement (91%); (3), 53 of 54 SLE-DAH patients received glucocorticoid therapy, 37 (69%) received methylprednisolone shock therapy, (4) the nosocomial mortality of SLE-DAH patients was 20. 4%. In the death group, there were statistical differences in age and course of disease, and the incidence of infection and mechanical ventilation were higher, or associated with poor prognosis. (5) compared with SLE-NDAH patients, the two groups had a SLEDAI score in the course of the disease. There were significant differences in hospital mortality (20% vs 7%), and there were skin and mucosal lesions (70.4% vs 49.0%), hematological diseases (87% vs 43.8%), thrombocytopenia (72.2% vs 25.5%) and renal lesions (90.7% vs 62.9%) in patients with SLE-DAH. The positive rate of anti ds-DNA antibody was higher (55.6%vs 42.6%), and there was statistical difference (P0.05); (6). With the increase of clinical knowledge, the mortality of SLE-DAH decreased (50% vs 10%, P0.05). Conclusion the positive rate of ds-DNA is a serious complication of SLE, and no hemoptysis is found in the clinical manifestation. Attention should be paid to the diagnostic value of bronchoscopy. Active glucocorticoids, immunosuppressants can improve prognosis. Age of onset, course of disease with infection, mechanical ventilation and poor prognosis were related. Improving clinical knowledge can improve the prognosis of SLE-DAH.
【學(xué)位授予單位】：北京協(xié)和醫(yī)學(xué)院
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2015
【分類號(hào)】：R593.241

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