弥漫性结缔组织病合并弥漫性肺泡出血的临床研究

发布时间：2018-03-28 17:20

本文选题：结缔组织病　切入点：系统性红斑狼疮　出处：《广西医科大学》2014年硕士论文

【摘要】：目的研究分析30例弥漫性结缔组织病（CTD）合并弥漫性肺泡出血（DAH）患者的临床资料，总结其临床表现及辅助检查特点，探讨治疗与预后的关系，，提高对该疾病的认识。方法收集2006年1月至2013年12月广西医科大学第一附属医院住院确诊的30例CTD合并DAH患者的临床资料，同时按照年龄、性别分层后，以1：2的比例从同期住院诊断为CTD的患者中随机抽取60位病例资料完整的患者作为对照组，即非DAH组。回顾性分析30例CTD合并DAH患者的临床特点、诊疗经过及预后，并与同期住院的非DAH组60例患者资料进行比较。结果 1、在3932例住院SLE患者中有20例患者合并DAH，发病率为0.51%，虽经积极治疗，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，发病率为3.52%，其中死亡6例，病死率60%。SLE-DAH组相较于AASV-DAH组女性患者多（90%比40%，P=0.004），发生DAH时年龄小且发病率低（22.9±14.27岁比48.8±19.11岁，P=0.000），但两组比较病死率之间的差异无统计学意义（70%比60%，P=0.584）。 2、所有DAH患者均出现咳嗽，其中8例患者（26.67%）病程中无咳血丝痰或咯血。其他常见症状及体征有低氧血症（96.67%）、胸闷（96.67%）、肺部Up音（93.33%）、呼吸困难（86.67%）、胸痛（13.33%）。 3、DAH组患者发热、水肿、乏力、浆膜腔积液、肾损害、消化道受累、血液系统损害等出现的比例高于非DAH组。SLE-DAH患者神经系统受累发生率高于AASV-DAH患者（40%比0%，P=0.02）。所有患者均有3个或3个以上系统受累，且肾脏损害发生率为100%。 4、DAH组血红蛋白及血小板减少的比例高于对照组。两组间尿蛋白阳性率无差异，但DAH组管型尿阳性率显著高于对照组。血清转氨酶、肌酐、肌酸激酶升高者比例DAH组均明显高于对照组。DAH组患者低钙血症发生率高于非DAH组，且低血钙程度较重（1.86±0.25比2.07±0.21，P=0.000）。 5、SLE合并DAH患者发热、浆膜腔积液的发生率明显高于未出现DAH的SLE患者，且更容易合并有肾脏、血液及消化道等多系统损害，血肌酐升高的发生率高，血钙值较对照组明显下降；发生DAH时SLEDAI评分为14~31分，表现为中重度狼疮活动，且明显高于对照组（21±4.65比16.62±1.75，P=0.001）。 6、CTD合并DAH患者肾脏病变有：系膜增生（85.71%），新月体形成（35.71%），肾小球硬化（35.71%），肾间质纤维化（7.14%）。其中8例SLE患者肾脏病理类型分别为：IV型（37.5%）、V型（37.5%）、III型（12.5%）、V+IV（12.5%）。其中肾脏病理类型为IV型的3例SLE合并DAH患者均存活，而其他3种肾脏病理类型的患者均死亡。 7、将行MP1g/d及MP0.5g/d冲击治疗的患者分别与未行激素冲击治疗的患者相比较，发现前者生存率明显高于未行激素冲击治疗的患者（66.7%比15.4%，P=0.013），差异有统计学意义，而后者生存率与未行激素冲击治疗患者相比无明显差异（28.6%比15.4%，P=0.489）。接受环磷酰胺冲击的患者生存率明显高于未接受环磷酰胺冲击治疗的患者（60%比7.1%，P=0.005）。 8、29例接受治疗的DAH患者中13例诊治科室为风湿免疫科，其余16例患者诊治科室分别为ICU、儿科、肾内科、呼吸科及皮肤科。通过比较专科及非专科治疗患者治疗方案的选择及预后情况，发现风湿免疫科行MP冲击、CTX冲击，及MP+CTX联合冲击治疗的比例明显高于其他科室，而风湿病专科治疗的死亡率则明显低于非专科治疗（38.5%比87.5%，P=0.006），即专科治疗更积极且预后更好。 9、死亡组低钙血症的发生率明显高于存活组，血小板数值明显低于存活组。存活组接受MP1g/d、环磷酰胺冲击治疗的比例明显高于死亡组，而死亡组机械通气率明显高于存活组（P0.05）。将以上观察指标作为自变量纳入模型，进一步采用Logistic回归分析，未发现以上因素为死亡相关危险因素。结论 1、CTD合并DAH患者发热、水肿、乏力出现的几率大于非DAH患者，且易合并肾脏、消化道及多系统受累；病程中可无咳血丝痰或咯血。 2、CTD合并DAH患者管型尿、肝肾功能损害、肌酶升高的发生率高，低钙血症的发生率高，且程度重。 3、死亡组患者低钙血症发生率高，血小板平均值低，机械通气率高，但均未得出以上因素为死亡相关危险因素。 4、MP1g/d冲击治疗、环磷酰胺冲击治疗可提高患者生存率，且MP1g/d治疗效果优于MP0.5g/d。 5、风湿病专科治疗有利于改善CTD合并DAH患者预后。
[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.

【学位授予单位】：广西医科大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R593.2;R563

【参考文献】