本文選題：結締組織病　切入點：系統(tǒng)性紅斑狼瘡　出處：《廣西醫(yī)科大學》2014年碩士論文

【摘要】：目的研究分析30例彌漫性結締組織�。–TD）合并彌漫性肺泡出血（DAH）患者的臨床資料，總結其臨床表現(xiàn)及輔助檢查特點，探討治療與預后的關系，，提高對該疾病的認識。 方法收集2006年1月至2013年12月廣西醫(yī)科大學第一附屬醫(yī)院住院確診的30例CTD合并DAH患者的臨床資料，同時按照年齡、性別分層后，以1：2的比例從同期住院診斷為CTD的患者中隨機抽取60位病例資料完整的患者作為對照組，即非DAH組�；仡櫺苑治�30例CTD合并DAH患者的臨床特點、診療經(jīng)過及預后，并與同期住院的非DAH組60例患者資料進行比較。 結果 1、在3932例住院SLE患者中有20例患者合并DAH，發(fā)病率為0.51%，雖經(jīng)積極治療，仍有14例患者死亡，病死率70%；在284例住院AASV患者中10例合并DAH，發(fā)病率為3.52%，其中死亡6例，病死率60%。SLE-DAH組相較于AASV-DAH組女性患者多（90%比40%，P=0.004），發(fā)生DAH時年齡小且發(fā)病率低（22.9±14.27歲比48.8±19.11歲，P=0.000），但兩組比較病死率之間的差異無統(tǒng)計學意義（70%比60%，P=0.584）。 2、所有DAH患者均出現(xiàn)咳嗽，其中8例患者（26.67%）病程中無咳血絲痰或咯血。其他常見癥狀及體征有低氧血癥（96.67%）、胸悶（96.67%）、肺部Up音（93.33%）、呼吸困難（86.67%）、胸痛（13.33%）。 3、DAH組患者發(fā)熱、水腫、乏力、漿膜腔積液、腎損害、消化道受累、血液系統(tǒng)損害等出現(xiàn)的比例高于非DAH組。SLE-DAH患者神經(jīng)系統(tǒng)受累發(fā)生率高于AASV-DAH患者（40%比0%，P=0.02）。所有患者均有3個或3個以上系統(tǒng)受累，且腎臟損害發(fā)生率為100%。 4、DAH組血紅蛋白及血小板減少的比例高于對照組。兩組間尿蛋白陽性率無差異，但DAH組管型尿陽性率顯著高于對照組。血清轉氨酶、肌酐、肌酸激酶升高者比例DAH組均明顯高于對照組。DAH組患者低鈣血癥發(fā)生率高于非DAH組，且低血鈣程度較重（1.86±0.25比2.07±0.21，P=0.000）。 5、SLE合并DAH患者發(fā)熱、漿膜腔積液的發(fā)生率明顯高于未出現(xiàn)DAH的SLE患者，且更容易合并有腎臟、血液及消化道等多系統(tǒng)損害，血肌酐升高的發(fā)生率高，血鈣值較對照組明顯下降；發(fā)生DAH時SLEDAI評分為14~31分，表現(xiàn)為中重度狼瘡活動，且明顯高于對照組（21±4.65比16.62±1.75，P=0.001）。 6、CTD合并DAH患者腎臟病變有：系膜增生（85.71%），新月體形成（35.71%），腎小球硬化（35.71%），腎間質纖維化（7.14%）。其中8例SLE患者腎臟病理類型分別為：IV型（37.5%）、V型（37.5%）、III型（12.5%）、V+IV（12.5%）。其中腎臟病理類型為IV型的3例SLE合并DAH患者均存活，而其他3種腎臟病理類型的患者均死亡。 7、將行MP1g/d及MP0.5g/d沖擊治療的患者分別與未行激素沖擊治療的患者相比較，發(fā)現(xiàn)前者生存率明顯高于未行激素沖擊治療的患者（66.7%比15.4%，P=0.013），差異有統(tǒng)計學意義，而后者生存率與未行激素沖擊治療患者相比無明顯差異（28.6%比15.4%，P=0.489）。接受環(huán)磷酰胺沖擊的患者生存率明顯高于未接受環(huán)磷酰胺沖擊治療的患者（60%比7.1%，P=0.005）。 8、29例接受治療的DAH患者中13例診治科室為風濕免疫科，其余16例患者診治科室分別為ICU、兒科、腎內(nèi)科、呼吸科及皮膚科。通過比較�？萍胺菍？浦委熁颊咧委煼桨傅倪x擇及預后情況，發(fā)現(xiàn)風濕免疫科行MP沖擊、CTX沖擊，及MP+CTX聯(lián)合沖擊治療的比例明顯高于其他科室，而風濕病�？浦委煹乃劳雎蕜t明顯低于非專科治療（38.5%比87.5%，P=0.006），即�？浦委煾e極且預后更好。 9、死亡組低鈣血癥的發(fā)生率明顯高于存活組，血小板數(shù)值明顯低于存活組。存活組接受MP1g/d、環(huán)磷酰胺沖擊治療的比例明顯高于死亡組，而死亡組機械通氣率明顯高于存活組（P0.05）。將以上觀察指標作為自變量納入模型，進一步采用Logistic回歸分析，未發(fā)現(xiàn)以上因素為死亡相關危險因素。 結論 1、CTD合并DAH患者發(fā)熱、水腫、乏力出現(xiàn)的幾率大于非DAH患者，且易合并腎臟、消化道及多系統(tǒng)受累；病程中可無咳血絲痰或咯血。 2、CTD合并DAH患者管型尿、肝腎功能損害、肌酶升高的發(fā)生率高，低鈣血癥的發(fā)生率高，且程度重。 3、死亡組患者低鈣血癥發(fā)生率高，血小板平均值低，機械通氣率高，但均未得出以上因素為死亡相關危險因素。 4、MP1g/d沖擊治療、環(huán)磷酰胺沖擊治療可提高患者生存率，且MP1g/d治療效果優(yōu)于MP0.5g/d。 5、風濕病�？浦委熡欣诟纳艭TD合并DAH患者預后。
[Abstract]:Objective to study and analyze the clinical data of 30 patients with diffuse connective tissue disease (CTD) complicated with diffuse alveolar hemorrhage (DAH), summarize the clinical manifestations and the characteristics of auxiliary examination, explore the relationship between treatment and prognosis, and improve the understanding of the disease.
Methods from January 2006 to December 2013 from the First Affiliated Hospital of Guangxi Medical University hospital diagnosed 30 cases of CTD patients with DAH clinical data, at the same time, according to age, gender stratification, with the ratio of 1:2 from the same hospital diagnosed CTD patients were randomly selected in 60 cases all patients as control group, non DAH group retrospectively. Analysis of clinical characteristics of 30 cases of CTD patients with DAH, treatment and prognosis, and hospitalization of non DAH group of 60 patients were compared.
Result
1, in 3932 cases of hospitalized patients with SLE in 20 patients with DAH, the incidence rate was 0.51%, although after active treatment, there are still 14 patients died, the mortality rate was 70%; in 284 cases of hospitalized patients with AASV in 10 patients with DAH, the incidence rate was 3.52%, of which 6 cases died, the mortality rate compared with the 60%.SLE-DAH group in the AASV-DAH group of female patients (90% more than 40%, P=0.004), DAH age and the incidence rate is low (22.9 + 14.27 to 48.8 + 19.11 years, P=0.000), but there was no significant difference between the two groups of mortality (70% vs 60%, P=0.584).
2, all DAH patients had cough. 8 patients (26.67%) had no cough, bloody sputum or hemoptysis during the course of the disease. Other common symptoms and signs were hypoxemia (96.67%), chest distress (96.67%), lung Up tone (93.33%), dyspnea (86.67%), chest pain (13.33%).
3, DAH group of patients with fever, fatigue, edema, effusion, renal damage, gastrointestinal involvement, the proportion of the blood system damage was higher than that of DAH group.SLE-DAH patients with nervous system involvement was higher than that of AASV-DAH patients (40% vs 0%, P=0.02). All patients had 3 or more than 3 system involvement occurred the rate of renal damage and 100%.
4, DAH group of hemoglobin and platelet reduction ratio is higher than the control group. There was no difference between two groups of urine protein positive rate, but the DAH group cylindruria positive rate was significantly higher than the control group. Serum aminotransferase, creatinine and creatine kinase increased the proportion of DAH group were significantly higher than that in.DAH group were lower than non DAH incidence of hypocalcemia group, and severe hypocalcemia (1.86 + 0.25 to 2.07 + 0.21, P=0.000).
5, SLE and DAH in patients with fever, serous effusion was significantly higher than that in SLE patients without DAH, and more easily with the kidney, blood and gastrointestinal tract and other damage, serum creatinine increased the incidence of high blood calcium decreased significantly than that of the control group; DAH SLEDAI score of 14~31, for moderate to severe lupus activity, and significantly higher than the control group (21 + 4.65 to 16.62 + 1.75, P=0.001).
In 6, CTD in patients with DAH nephropathy: mesangial hyperplasia (85.71%), crescent formation (35.71%), glomerular sclerosis (35.71%), renal interstitial fibrosis (7.14%). Among the 8 cases of SLE patients with renal pathological types were: type IV (37.5%), V (37.5%), III (12.5%), V+IV (12.5%). The renal pathological types as type IV in 3 cases of SLE patients with DAH were alive, and 3 other types of renal pathology of the patients were dead.
7, by MP1g/d and MP0.5g/d shock treated patients respectively and without corticosteroid therapy were compared, found that the survival rate was significantly higher than non steroid treated patients (66.7% vs 15.4%, P=0.013), the difference was statistically significant, then the survival rate and without hormonal impact and no significant differences in treatment of patients (28.6% vs 15.4%, P=0.489). The patients received cyclophosphamide impact survival rate was significantly higher than untreated CTX treated patients (60% vs 7.1%, P=0.005).
8,29 DAH patients who received treatment in the diagnosis and treatment of 13 cases of the Department of Rheumatology, the remaining 16 cases were Department of Pediatrics, ICU, renal medicine, Department of respiration and Department of dermatology. Through the selection and prognosis were compared between the specialist and non specialist treatment in patients with rheumatic disease were found, free MP impact, the impact of CTX and MP+CTX pulse therapy was significantly higher than the proportion of other departments, and specialist treatment rheumatism mortality was significantly lower than that of non specialist treatment (38.5% vs 87.5%, P=0.006), which is specialized for more aggressive treatment and the prognosis is better.
9, the death group the incidence of hypocalcemia was significantly higher than the survival group, platelet count was significantly lower in the survival group. The survival group received MP1g/d, cyclophosphamide pulse therapy was significantly higher than the proportion of death group and death group, mechanical ventilation rate significantly higher than the survival group (P0.05). The observation index as independent variables into the model, further using Logistic regression analysis without the above factors as risk factors related to mortality.
conclusion
1, the incidence of fever, edema and fatigue in patients with CTD combined with DAH is greater than that in non DAH patients, and is easy to merge with kidney, gastrointestinal tract and multiple system involvement. There is no cough, bloody sputum or hemoptysis in the course of disease.
2 CTD patients with DAH urinary tube, liver and kidney dysfunction, muscle enzymes increased with high incidence rate, high incidence of hypocalcemia, and the degree of weight.
3, the high incidence of hypocalcemia in death group, mean platelet value is low, the mechanical ventilation rate is high, but did not get the above factors for death related risk factors.
4, MP1g/d impact therapy, cyclophosphamide impact therapy can improve the survival rate of patients, and the effect of MP1g/d is better than MP0.5g/d..
5, rheumatic disease treatment is beneficial to improve the prognosis of CTD combined with DAH.

【學位授予單位】：廣西醫(yī)科大學
【學位級別】：碩士
【學位授予年份】：2014
【分類號】：R593.2;R563

【參考文獻】

相關期刊論文 前3條

1 鮑浩;李世軍;;免疫吸附治療系統(tǒng)性紅斑狼瘡合并彌漫性肺泡出血[J];腎臟病與透析腎移植雜志;2006年06期

2 趙明輝,章友康,劉玉春,劉娜,王海燕;抗中性粒細胞胞漿抗體相關小血管炎的系統(tǒng)表現(xiàn)[J];中華內(nèi)科雜志;2000年01期

3 徐巧蓮;萬獻堯;;ICU患者低鈣血癥發(fā)生率調(diào)查與危險因素分析[J];醫(yī)學與哲學(B);2012年03期

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