系统性红斑狼疮合并弥漫性肺泡出血临床分析

目的 总结系统性红斑狼疮(SLE)并发弥漫性肺泡出血(DAH)的临床特点和治疗效果.方法 收集1 247例住院SLE患者的临床资料,其中21例并发DAH(SLE并发DAH组),1 226例SLE(单纯SLE组),回顾性分析患者的临床表现、诊治经过和预后.结果 DAH是SLE少见的并发症,发生率为1.7%,病死率为38.1%,两组患者系统性红斑狼疮疾病活动评分(SLEDAI)、血WBC计数、血小板计数(PLT)、血IgG、血红蛋白、血氧饱和度、24 h尿蛋白、补体C3水平和抗双链DNA(抗dsDNA)阳性率比较,差异均有统计学意义(P<0.05);两组患者呼吸困难发生率、咳嗽咳痰率、咯血痰中带血发生率肺部阴影率、血管炎样皮疹率、肺动脉高压发生率比较,差异均有统计学意义(P<0.05);支气管肺泡灌洗治疗能够提高DHA患者存活率和急性起病率间比较,差异均有统计学意义(P<0.01).结论 DAH 是SLE严重并发症,与疾病活动有关.最常见的表现为急性呼吸困难和新发的肺部浸润,及早行支气管镜检查、支气管肺泡灌洗治疗对及时诊治至关重要.     

系统性红斑狼疮并弥漫性肺泡出血的临床研究(附2例报道)

目的提高对系统性红斑狼疮(SLE)并弥漫性肺泡出血(DAH)的认识。方法报道2例SLE并DAH的临床资料,并复习相关文献。结果通过分析病例,总结SLE并DAH的临床特点、发病机制、诊断标准、治疗手段和预后。结论DAH为SLE少见而致命的并发症,发热、咯血、呼吸困难、血红蛋白下降等临床表现无特异性。影像学显示双肺弥漫性浸润影。血性支气管肺泡灌洗液内见含铁血黄素巨噬细胞。肺组织学呈弥漫性肺泡出血。早期足量激素和免疫抑制剂治疗可改善其预后。     

系统性红斑狼疮合并弥漫性肺泡出血的临床疗效分析

目的:总结和分析临床治疗系统性红斑狼疮(Systemic lupus erythematosus,SLE)合并弥漫性肺泡出血的主要特点及其治疗手段,供临床诊治该病提供参考。方法:采用回顾性分析研究方法对我院2009年7月至2013年7月间收治的系统性红斑狼疮合并弥漫性肺泡出血患者30例的病历记录资料做总结分析,对该疾病的主要临床特点、诊断要点及其治疗方法做分析。结果:本次研究所收集的30例患者均为女性,平均年龄34.3岁,临床表现以咳嗽咳痰、咯血或咯粉红色泡沫痰、低氧血症伴或不伴呼吸困难、血红蛋白降低、红细胞压积减小为主,多数患者可同时合并有狼疮性肾炎;诊断主要是建立在系统性红斑狼疮基础上,结合临床表现和肺部X线片机CT检查可确诊;治疗上主要使用泼尼松龙联合环磷酰胺做冲击治疗,同时通过呼吸机来改善呼吸状况,支气管镜肺泡灌洗的应用大大提高了治疗疗效,同时可采用成分输血及其静脉输注丙种免疫球蛋白做支持治疗;该病预后较差,本组30例患者4例死于呼吸衰竭。结论:系统性红斑狼疮合并弥漫性肺泡出血是SLE一个极为严重的并发症,发病不是很多见,但后果比较严重,很多患者极容易因为呼吸衰竭而死亡,是SLE一个急危重症,通过呼吸机的辅助呼吸及其他辅助治疗可以有效提高患者生存率,改善预后。     

系统性红斑狼疮合并肺泡出血10例临床分析

目的总结SLE合并肺泡出血的临床特点和疗效分析,提高对该病的认识。方法回顾性分析我院2010年2月~2017年4月确诊SLE合并DAH的10例临床资料,并进行统计学分析。结果 (1)10例SLE-DAH患者中,男1例,女9例,平均年龄(27.3±11.6)岁,平均病程(18.2±25.4)个月。(2)3例以DAH表现首发。最常见的症状是气促或呼吸困难、低氧血症,其余依次为咳嗽、发热、咯血、痰中带血;24~48 h内血红蛋白平均下降(20.2±9.5)g/L。最常见的肺外受累为肾脏、浆膜腔;(3)SLEDAI评分平均(24.8±10.1)分,其中90%处于疾病重度活动期;(4)10例患者均接受了糖皮质激素治疗,4例予甲基泼尼松龙冲击治疗;8例予丙种免疫球蛋白冲击,6例使用环磷酰胺抑制免疫,4例予机械通气,1例行血浆置换,病死率为80%。结论 SLE合并DAH的死亡率高,疾病活动度高易发生。积极的糖皮质激素、免疫抑制剂是治疗的主要方法。     

支气管肺泡灌洗对系统性红斑狼疮合并弥漫性肺泡出血抢救价值的探讨

目的 探讨支气管肺泡灌洗在系统性红斑狼疮(SLE)合并弥漫性肺泡出血(DAH)抢救治疗中的应用价值.方法 将21例SLE合并DAH患者随机分成两组,分别给予两种抢救措施,A组给予注射用甲泼尼松琥珀酸钠1000 mg冲击治疗3 d,B组给予注射用甲泼尼松琥珀酸钠1000 mg冲击治疗3 d辅助支气管肺泡灌洗1次.观察两组患者治疗前后二氧化碳分压(PaCO2)、氧分压、氧饱和度及呼吸困难程度勃氏评分(BS)的变化.结果 治疗前后比较,除A组PaCO2改善其他指标差异无统计学意义(P>0.05),B组的PaCO2以及两组的氧分压和氧饱和度均有显著性改善(均P<0.05).治疗后两组间比较PaCO2差异无统计学意义(P>0.05),B组氧分压和氧饱和度改善程度显著高于A组(均P<0.05);经过治疗两组患者Bs显著下降(均P<0.01),而且B组下降程度大于A组(P<0.05).结论 支气管肺泡灌洗联合大剂量注射用甲泼尼松琥珀酸钠冲击治疗,可以显著改善SLE合并DAH患者急性期低氧血症及呼吸困难.

Abstract：

Objective To explore the value of bronchoalveolar lavage as an emergency treatment for systemic lupus erythematosus(SLE)patients with concurrent diffuse alveolar hemorrhage(DAH).Methods A total of 21 SLE plus DAH patients were divided randomly into 2 groups.The patients in Group A received methylprednisolone 1000 mg/d for 3 days while those in Group B methylprednisolone 1000 mg/d for 3 days in combination with a bronchoalveolar lavage.Partial pressure of carbon dioxide(PaCO2),partial pressure of oxygen(PaO2),oxygen saturation(SaO2)and Borg scale(BS)for quantitative evaluation of dyspnea were recorded before and after treatment.Results Except for PaCO2,all other parameters of blood gas analysis in all patients in Group A(P>0.05)were better after treatment than before(all P<0.05).After treatment,PaO2 and SaO2 in Group B were higher than those in Group A(all P<0.05),but PaCO2 was not markedly changed(P>0.05).BS decreased significantly in both groups(both P<0.01).And more decrease was observed in Group B as compared with Group A after treatment(P<0.05).Conclusion Bronchoalveolar lavage plus a high-dose implosive therapy of methylprednisolone may alleviate hyoxemia and dyspnia in acute period of SLE complicated with DAH.     

系统性红斑狼疮合并弥漫肺泡出血17例临床分析

目的 结系统性红斑狼疮(SEE)合并弥漫肺泡出血(DAH)的临床特点.方法 回顾性分析17例SLE合并DAH患者的临床表现、实验室检查、诊治和转归.结果 SLE合并DAH主要表现为低氧血症(100%)、呼吸困难(88%)、咳嗽(88%)、发热(82%)和咯血(71%).大部分(94%)患者同时合并肾脏受累.近60%合并肺部感染.血红蛋白平均下降(32±11.3)g/L.SLE疾病活动指数平均(17 ±10)分.17例患者全部接受激素治疗,13例并用免疫抑制剂.死亡组机械通气率高于存活组(P<0.05).结论 DAH是SEE少见而危重的并发症,与SLE疾病活动有关.常合并狼疮性肾炎.并非所有患者均有咯血表现.     

系统性红斑狼疮合并弥漫性出血性肺泡炎及急性横贯性脊髓炎一例

报告1例临床少见的系统性红斑狼疮合并弥漫性出血性肺泡炎及急性横贯性脊髓炎病例，提示早期诊断，联合应用激素、免疫抑制剂、免疫球蛋白及鞘内注射，能够有效的控制病情。并检索复习国内、外相关文献，讨论其并发症的临床表现、诊断、治疗与预后，以提高临床医生对其的认识。     

系统性红斑狼疮并发弥漫肺泡出血5例临床观察

弥漫肺泡出血（DAH）是系统性红斑狼疮（SLE）的并发症之一，临床上虽然少见．但病情重，病死率高，并且临床表现不特异．常常漏诊、误诊。其疗效和预后多取决于及时的诊治。国内报道不多。我院收治5例，现报告如下。     

以弥漫性肺泡出血为主要首发表现的儿童系统性红斑狼疮1例

患者,男,14岁,因发现面部红斑,胸闷、胸痛伴发热2月,加重1周于2010 年5月31日入院,患者入院前2个月无明显诱因出现面部蝶形红斑,无瘙痒及疼痛感,伴胸闷、胸痛,为双侧对称性胸痛,无心前区疼痛,偶有咳嗽,无咯痰,伴低热,无关节及肌肉疼痛等.     

17例弥漫性肺泡出血临床分析

目的提高对弥漫性肺泡出血的诊断及治疗水平。方法分析1998年7月至2008年5月期间17例因弥漫性肺泡出血入住RICU患者的临床资料。结果 17例患者中确诊为韦格纳肉芽肿6例,显微镜下多血管炎3例,系统性红斑狼疮4例,Goodpasture综合征1例,二尖瓣狭窄2例,特发性肺嗜铁血黄素沉着症1例。临床表现:发热76.4%(13/17),咯血47%(8/17),贫血100%(17/17),呼吸困难100%(17/17),低氧血症100%(17/17),白细胞升高76.4%(13/17)。血红蛋白(78.0±2.4)g/L。胞浆型中性粒细胞胞浆抗体(C-ANCA)滴度1∶(46.0±3.7);核周型中性粒细胞胞浆抗体(p-ANCA)滴度1∶(108.0±16.1)。支气管肺泡灌洗液呈血性35.3%(6/17);噬含铁血黄素巨噬细胞计数阳性100%(17/17)。胸部CT呈弥漫性肺泡浸润实变影、磨玻璃影、网格影100%(17/17),15例呈两肺弥漫性阴影,2例呈右肺弥漫性阴影。死亡率29.4%(5/17)。结论弥漫性肺泡出血是危及生命的危重症,咯血、呼吸困难、贫血及胸部X线影像为诊断弥漫性肺泡出血提供线索。糖皮质激素和免疫抑制剂是治疗弥漫性肺泡出血的有效方法。     

弥漫性结缔组织病并发弥漫性肺泡出血28例临床分析

目的总结弥漫性结缔组织病(DCTD)并发弥漫性肺泡出血(DAH)的临床特点和治疗效果。方法回顾性分析百色市人民医院2009年1月—2012年12月确诊的DCTD合并DAH患者28例的临床资料。结果DCTD合并DAH的患者中,以显微镜下多血管炎(MPA)居多占17例,其次为系统性红斑狼疮(SLE)占8例,韦格纳肉芽肿(WG)2例,类风湿性关节炎(RA)1例。DAH主要表现为呼吸困难、低氧血症、进行性贫血,CT示新发的肺部浸润影。DAH患者发生感染21例(75.0%);肺部感染占首位,共有19例(90.5%)。28例患者全部接受了激素和免疫抑制剂治疗,其中予甲泼尼龙冲击治疗20例,血浆置换15例,行机械通气11例,行透析13例。最终存活10例(35.7%)。死亡18例(64.3%)。比较2组存活与死亡特点,发现存活组血浆置换治疗率高于死亡组(P<0.05),机械通气率低于死亡组(P<0.05)。结论 DAH是DCTD的一种严重并发症,病死率高,与疾病活动有关。呼吸困难、咯血、贫血及肺部CT为DAH诊断提供线索。糖皮质激素、免疫抑制剂联合血浆置换是治疗DAH有效方法。     

系统性红斑狼疮合并自发性肾上腺出血1例

A 58-year-old female was referred to our department with intermittent suffocation for 1.5 years, aggravated for a month. 1.5 years before she developed oral ulcer, raynaud phenomenon, proteinuria, bilateral pleural effusion, ANA and anti-dsDNA positive. This patient was diagnosed with systemic lupus erythematosus (SLE). After given hormones, hydroxychloroquine sulfate (HCQ), her symptom relieved soon. The patient stopped her pills 1 year ago. One month ago, she had chest tightness, increased urine foam, and suffered from oliguria. Her admission medical examination: blood pressure (BP) 130/80 mmHg, conjunctiva pale, and lower lung breath sounds reduced. There was no tenderness, rebound pain and abdominal muscle tension in the abdomen. Liver and spleen rib inferior, mobile dullness negative, and lower extremity edema. Blood routine tests were performed with hemoglobin (HGB) 57 g/L. Urine routine: BLD (3+). 24-hour urinary protein 3.2 g. serum albumin 20.5 g/L, C-reactive protein (CRP) 12.85 mg/L, erythrocyte sedimentation rate (ESR) 140 mm/h. Antinuclear antibody (ANA) (H)1 ∶10 000;, anti-dsDNA antibody 1 ∶3 200;, anti-Smith antibody, anti-U1-snRNP / Sm antibody were positive, blood complement 3(C₃) 0.43 g/L, complement 4(C₄) 0.07 g /L. Anticardiolipin antibody (ACL), anti-β2-GP1;, lupus anticoagulant (LA) were negative, HRCT suggested bilateral medial pleural cavity product liquid. Admission diagnosis: SLE lupus nephritis, anemia, pleural effusion, and hypoproteinemia. We treated her with methylprednisolone 1 000 mg×3 d;, late to 48 mg/d and cyclophosphamide 1.0 g, HCQ 0.2 g bid, gamma globulin 10 g×5 d. Day 2 of treatment;, this patient developed acute right upper quadrant pain, not accompanied by nausea, vomiting, blood stool and diarrhea. Antipyretic antispasmodic treatment was invalid, after the morning to ease their own abdominal pain. Day 4 of treatment, daytime blood HGB 77 g/L. Bilateral renal vascular ultrasound: bilateral renal artery blood flow velocity was reduced. The abdominal pain of the above symptoms recurred at night, BP was 120/80 mmHg, and no positive signs were found on abdominal examination. No abnormality was found in the vertical abdominal plain film. Blood routine examination: HGB 53 g/L, Plasma D dimer 2 515 μg/L;, amylase in hematuria was normal, the stool occult blood was negative. Abdominal computed tomography (CT): normal structure of right adrenal gland disappeared, irregular mass shadow could be seen in adrenal region, CT value was about 50 HU. Morphological density of left adrenal gland was not abnormal. The retroperitoneum descended along the inferior vena cava to the right iliac blood vessel and showed a bolus shadow. The density of some segments increased. The lesion involved the right renal periphery and reached the left side of abdominal aorta. Most lesions surrounded the inferior vena cava, the right renal vein and part of the small intestine. The boundary between the upper lesion and the vena cava was unclear. Iodine-containing contrast agent was taken orally. No sign of contrast agent overflowing was found in the abdominal cavity. Hematoma and exudative changes were considered in retroperitoneum. Conclusion of contrast-enhanced ultrasound of blood vessels: The retroperitoneal inferior vena cava (volume 3.5 cm×3.5 cm×1.5 cm) was hypoechoic and had no blood flow lesion. The adrenal gland had a high possibility of origin. Left renal vein thrombosis extended to inferior vena cava. According to the above data;, it was analyzed that the cause of retroperitoneal hematoma of the patient was left adrenal vein thrombosis caused by hypercoagulable state, which led to vascular rupture and hemorrhage caused by increased vascular pressure in adrenal gland. Therefore, on the basis of continuing to actively treat the primary disease, and on the basis of dynamic observation of no active hemorrhage for 3 days, the anticoagulant therapy was continued with 10 mg/d of apixaban. Clinical symptoms were gradually eased, HGB did not decrease. Two weeks later, the ultrasonic examination showed that the irregular cluster hypoechoic range behind the inferior vena cava was significantly smaller than that before (1.8 cm×1.2 cm×0.7 cm). Abdominal CT examination after 1 month showed that there was no abnormal morphological density of bilateral adrenal glands and basic absorption of retroperitoneal exudation. Adrenal hemorrhage is uncommon. SLE with adrenal hemorrhage is rarer. In SLE patients;, especially those complicated with APS, if abdominal pain accompanied by HGB decrease occurs, except after gastrointestinal hemorrhage, the possibility of adrenal hemorrhage should be warned.     

系统性红斑狼疮伴肝内多发出血1例报道

患者女,41岁.既往有类风湿关节炎、系统性红斑狼疮病史,无明显诱因感上腹部疼痛,向右背部放散,恶心未吐.查体：神智清,精神差,消瘦貌,四肝躯干散在多个狼疮样红斑,揭去皮屑,有带状出血点.     

系统性红斑狼疮合并消化道出血6例

系统性红斑狼疮(SLE)是一种多系统受累的自身免疫性疾病,累及胃肠道并以消化道出血为突出表现国内甚少报道。本文就我院1986～1996年内科收治的127例SLE患者中6例以消化道出血表现为主者进行临床分析。     

1例脂肪栓塞致弥漫性肺泡出血的救治体会

弥漫性肺泡出血是一种可导致呼吸衰竭的致命性的临床综合征,常常是发生在一系列疾病过程中的严重并发症[1]。本病临床中较为少见,大部分患者病情凶险,发病后短期内即死亡,诊断和治疗极为困难。我们收治了1例由脂肪栓塞致弥漫性肺泡出血的患者,经中西医积极治疗,抢救成功,     

以出血为首发症状的系统性红斑狼疮8例分析

以出血为首发症状的系统性红斑狼疮８例分析陈文华周文敬（延边医学院附属医院１３３０００）陈静（延吉市啤酒厂卫生所１３１３０１）系统性红斑狼疮（ＳＬＥ）较常见，以出血为第一表现，往往易误诊为原发性血小板减少性紫癜（ＩＴＰ）。本文分析４８例ＳＬＥ患者中，以...     

WONCA Online电子病例介绍——弥漫性肺泡出血

病例简介：患者，男，67岁，因严重的、持续性的呼吸困难4d就诊。患者自诉4d以来咳嗽，痰中带血，食欲减退，体重减轻。无发烧、畏寒症状，无胸痛、端坐呼吸、下肢肿胀等。无毒物接触史。既往因心动过速服用胺碘酮，无肺结核、房颤、心肌梗死、充血性心力衰竭等病史。     

系统性红斑狼疮并弥漫性血管内凝血1例的整体护理

<正>系统性红斑狼疮(ｓｙｓｔｅｍｉｃ ｌｕｐｕｓｅｒｙｔｈｅｍａｔｏｓｕｓ,ＳＬＥ)是一种累及全身多个系统的自身免疫性疾病,以年青女性多见。病因不明,可能与遗传、性激素、环境等多种因素有关,具有病程长,易复发,不能彻底根治等特点,临床治疗及护理均较为复杂。如果并发有感染、肾功能衰竭、弥     

儿童系统性红斑狼疮继发干燥综合征1例

系统性红斑狼疮(systemic lupus erythematosus,SLE)和干燥综合征(sjogren’s syndrome,SS)是临床常见的自身免疫性疾病,儿童期临床表现多不典型,缺乏特异性,且多不单独存在,临床误诊或漏诊率较高[1-2],两者并存时被命名为系统性红斑狼疮继发干燥综合征(SLE-SS)[3]。目前,国内对SLE-SS的临