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1.
目的:分析系统性红斑狼疮(SLE)合并可逆性后部脑病综合征(PRES)的临床特点、治疗及预后。方法:回顾性分析SLE合并PRES患者的临床表现、实验室检查及影像学资料,探讨其发病机制、危险因素、治疗及预后特点。结果:本研究共纳入16例SLE患者,女性14例、男性2例,平均年龄(22. 8±8. 5)岁,16例均有狼疮重度活动,SLE活动性指数为(20. 9±3. 7)分。发生PRES前15例(93. 8%)有高血压,12例(75%)合并肾功能不全、淋巴细胞计数低下、高脂血症,16例患者均使用激素或免疫抑制剂。临床症状有癫痫发作(16例,100%)、头痛(5例,31%)、视觉障碍(5例,31%),恶心呕吐(6例,38%)。CT及磁共振成像(MRI)显示病灶多位于双侧顶枕叶,也可累及颞叶、额叶、脑室、基底节、小脑及皮层。降压、抗癫痫和小剂量激素控制狼疮活动后症状可缓解。结论:SLE合并PRES是一种临床-影像综合征,发病与高血压、肾功能不全、狼疮重度活动等因素相关,临床表现有头痛、视力障碍、癫痫发作和恶心呕吐,脑部影像学表现为双侧顶枕区的皮层下血管源性水肿。SLE合并PRES时若能及时诊断并治疗,病变可逆,预后良好。  相似文献   

2.
目的分析孕产妇合并可逆性后部脑病综合征(posterior reversible encephalopathy syndrome,PRES)患者的临床影像特点,并与非孕产妇合并PRES患者进行比较。方法回顾性分析2002年10月至2012年12月在北京协和医院住院的12例孕产妇PRES患者的临床、影像表现及治疗和预后,并与同期收治的25例非孕产妇PRES进行比较。结果 12例孕产妇合并PRES患者平均年龄25.8岁(18~39岁)。9例发生在晚孕期,1例中孕期,2例产后。临床特征:12例患者就诊时血压均不同程度升高,平均收缩压164 mm Hg(140~180 mm Hg)。临床表现头痛10例,抽搐9例,不同程度意识障碍5例,视觉障碍5例。影像学特征:12例患者急性期行头颅磁共振成像(MRI)和(或)计算机断层扫描(CT)均可见局部脑组织水肿信号,大部分病灶位于双侧顶枕叶皮层下白质,其中顶叶病变11例,枕叶10例,颞叶7例,额叶6例,其他如脑干、小脑、基底节、丘脑病变者4例。治疗转归:12例患者均给予不同程度的降压、硫酸镁及甘露醇降颅压等治疗,10例孕期发病患者均给予急诊剖宫产终止妊娠。所有患者最终均好转出院,未遗留神经系统症状体征,复查影像学病灶明显减少或消失。与25例非孕产妇PRES比较,孕产妇PRES患者在发病年龄、临床表现、病灶分布上差异无统计学意义,但是预后相对更好。结论孕产妇合并PRES多发生在孕晚期或产后,与子痫好发时间及临床表现类似,及时影像学检查非常必要;孕产妇合并PRES的临床影像特征与非孕产妇PRES比较差异无统计学意义,但是治疗及时预后相对更好。  相似文献   

3.
目的:观察分析系统性红斑狼疮(SLE)患者合并假性肠梗阻(IPO)的临床特点及预后。方法:2004年1月至2014年4月南京军区南京总医院肾脏科住院确诊为SLE合并IPO患者30例,回顾性分析其临床表现、实验室检查结果、影像学特点、治疗及预后。结果:30例SLE合并IPO患者中,女性26例,男性4例,发生IPO时平均年龄(26.1±11.2)岁,SLE中位病程为27.5月,狼疮活动性指数(SLE-DAI)评分(11.2±7.4)分。6例有明确诱因(5例不洁饮食、1例上呼吸道感染)。IPO临床症状依次为恶心呕吐(76.6%)、腹痛(66.7%)、腹泻(56.7%)和腹胀(30%)。腹部平片及腹部CT可辅助诊断肠梗阻,阳性率75%,7例患者同时合并肾盂、输尿管积水。96.7%患者存在贫血,9例患者血小板减少,8例患者白细胞减少,4例直接Coombs试验阳性。96.7%的患者补体C3降低,ANA阳性率89.7%,抗ds-NDA抗体阳性率51.7%,抗心磷脂抗体阳性率30.4%,狼疮抗凝物阳性率43.8%,抗C1q抗体阳性率47.4%。29例患者接受激素为主[泼尼松0.6~1 mg/(kg·d)]的免疫抑制剂治疗(其中10例甲泼尼龙冲击治疗,11例丙种免疫球蛋白治疗),病情均好转出院,IPO好转中位数时间12d。24例患者长期随访(1~60月),2例死亡,4例行腹膜透析,5例反复发生IPO,至随访终点,肾脏完全缓解率25%,部分缓解率41.7%。结论:SLE累及消化道导致IPO临床相对罕见,但症状较重,容易误诊,早期识别,并及时给予免疫抑制剂治疗。  相似文献   

4.
目的总结系统性红斑狼疮(systemic lupus erythematosus,SLE)合并Evans综合征患者的临床特点。方法回顾性分析北京协和医院2004年1月至2012年7月SLE合并Evans患者的临床表现及实验室特点及治疗和预后。结果 SLE并发Evans综合征患者22例,占同期SLE住院患者3400例的0.65%。其中男3例,女19例,平均35.1岁(16~53岁)。22例患者中以血液系统受累[特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)或自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)]为首发表现的11例(50%),确诊SLE后诊断Evans综合征者6例,二者同时诊断的5例。SLE并发Evans综合征时,患者往往有多系统受累,表现为肾脏受累13例(59.1%),皮肤黏膜受累、关节炎各9例(40.9%),神经系统受累4例(18.2%),胃肠道、肺部受累各2例等。Evans综合征多发生于SLE活动期,患者平均狼疮活动指数评分(11.45±7.6)分(3~30分)。伴发其他结缔组织病5例(22.7%)。经激素联合免疫抑制剂治疗后,20例好转,2例无效者应用利妥昔单抗后好转。结论 SLE合并Evans综合征罕见,发生于SLE多系统受累及活动期。部分患者以ITP或AIHA为SLE首发表现,应及时筛查多种自身抗体,并定期随访密切观察,以期早期诊治。  相似文献   

5.
正可逆性后部脑病综合征(posterior reversible encephalopathy syndrome,PRES)最早于1996年由Hinchey等提出[1],是由各种病因引起,以头痛、呕吐、视物不清、癫痫、意识障碍等神经系统症状为主要表现的综合征,临床和神经影像学改变有可逆性。PRES多见于高血压、严重肾脏病、急性肾功能衰竭、免疫相关疾病、恶性肿瘤化疗和器官移植术后接受免疫抑制治疗等患者,高血压是其最常见  相似文献   

6.
目的 了解系统性红斑狼疮(SLE)合并乙型肝炎病毒(HBV)感染患者临床特点.方法 回顾性分析2003年2月至2011年6月在北京协和医院住院的40例合并HBV感染的SLE患者临床资料,并与性别、发病年龄相匹配的60例无HBV感染的SLE患者比较.统计学方法采用t检验和x2检验.结果 40例合并HBV感染者中男性4例,女性36例,平均年龄(32±13)岁;其中23例患者表现为慢性乙型肝炎,5例为HBV携带状态,12例为既往无症状感染.21例慢性乙型肝炎患者肝功能轻度异常主要为丙氨酸转氨酶升高,2例严重肝功能异常.临床表现合并HBV感染组和对照组比较,发热、肝脏受累、肾脏受累、血小板减少、巨细胞病毒感染更常见.肾病变主要为Ⅳ型或Ⅴ型狼疮肾炎,2例合并HBV相关性肾炎.血清乙型肝炎病毒表面抗原(HBsAg)阳性的SLE患者在接受免疫抑制剂治疗时同时使用拉米夫定抗病毒治疗.患者出院好转率为98% (39/40),1例预后不佳者有严重肝脏病变.结论 应重视SLE合并HBV感染患者临床特点:SLE合并HBV感染患者发热、肝脏受累、肾脏受累、血小板减少、巨细胞病毒感染者增多,可合并HBV相关性肾炎;肝脏病变严重者预后差;血清HBsAg阳性的SLE患者在接受免疫抑制剂治疗时应同时使用抗HBV药治疗.  相似文献   

7.
目的 提高临床医生对Churg-Srauss综合征(CSS)的认识.方法 分析5例经病理学确诊CSS患者的临床特点,并复习相关文献.结果 5例CSS患者均有不同病程的哮喘表现,3例有鼻炎或鼻窦炎,心脏受累和胃肠道受累各1例.5例患者外周血嗜酸粒细胞比例>10%.胸部影像学检查5例均表现肺部实变影,3例肺部实变影多变.所有病例经糖皮质激素或联合免疫抑制剂治疗后均缓解.结论 哮喘、外周血嗜酸粒细胞增多和肺部实变影多变是CSS的临床特点,糖皮质激素和免疫抑制剂是主要治疗药物.  相似文献   

8.
目的 了解系统性红斑狼疮(SLE)患者用药的基本情况,探讨规范化应用免疫抑制剂治疗的重要性,为改善SLE患者预后提供临床依据.方法 采用流行病学现况调查的研究方法,随机调查252例SLE患者,记录患者自发病以来的用药情况,并分析用药以及就诊情况等因素与肾脏损害之间的关系.结果 在252例SLE患者中药物治疗以糖皮质激素(99.2%)、免疫抑制剂(75.8%)、羟氯喹(61.5%)为主.免疫抑制剂以环磷酰胺为常用药(75.9%).应用免疫抑制剂6个月以上的患者仅有128例(50.8%).因惧怕不良反应而擅自停药仍为患者停用免疫抑制剂、羟氯喹的主要原因.通过单因素及多因素Logistic回归分析表明,规范应用激素、免疫抑制剂、羟氯喹治疗可以显著降低狼疮肾脏损害的发生率;并且应用免疫抑制剂治疗时间超过3个月,其肾损害以及肾功能不全的发生率明显降低(χ2=3.996,P<0.05;χ2=13.196,P<0.01),尤其是达到6个月以上的患者,这种差异更加明显(χ2=4.505,P<0.05;χ2=8.453,P<0.01).结论 SLE患者治疗主要是应用糖皮质激素联合免疫抑制剂、羟氯喹.尽早及规范应用糖皮质激素联合免疫抑制剂或羟氯喹治疗,可以显著降低狼疮肾脏损害的发生率.  相似文献   

9.
目的: 分析自身免疫性肝炎(AIH)、原发性胆汁性肝硬化(PBC)及原发性硬化性胆管炎(PSC)患者的临床特点及影像学特征.方法: 2003-10/2008-02在烟台毓璜顶医院消化内科住院的自身免疫性肝病患者43例, 排除其他原因引起肝脏损害. 其中AIH 12例, PBC 19例, PSC 12例. 对患者的临床及影像学资料进行回顾性分析, 观察药物治疗的效果.结果: 除PSC外, AIH多发于中年女性, 患者具有较高的转氨酶, PBC、PSC患者具有较明显的GGT、ALP升高. 临床表现上AIH、PBC、PSC黄疸的发生率分别为42%、80%、91%,皮肤瘙痒的发生率分别为38%、68%、79%.合理应用熊去氧胆酸和免疫抑制剂可使68%的PBC和AIH患者症状在6 mo内得到缓解、肝功能明显改善.结论: AIH的肝功能异常以转氨酶升高为主,PBC、PSC以胆汁淤积为主. 熊去氧胆酸和免疫抑制剂可改善绝大多数患者的症状和肝功能.  相似文献   

10.
目的探讨103例高血压发生脑出血患者的发病因素,为预防本病提供临床参考资料。方法选取2008年3月—2011年8月于我院住院治疗的高血压发生脑出血的患者103例,对其临床发病原因、出血部位等相关临床资料进行回顾性分析。结果 103例患者的诱因主要为大量饮酒13例(12.62%),剧烈运动11例(10.68%),长时间劳累9例(8.74%),情绪激动9例(8.74%),便秘7例(6.80%),无明显诱因54例(52.43%);发病危险因素可归纳为血压控制不良24例(23.30%),合并高脂血症29例(28.16%),合并糖尿病21例(20.39%),大量吸烟19例(18.45%),酗酒10例(9.71%)。结论大量饮酒、剧烈运动、长时间劳累、情绪激动、便秘是高血压脑出血(HICH)发病的主要诱因;血压控制不良、合并高脂血症、合并糖尿病、大量吸烟、酗酒是HICH的主要危险因素。  相似文献   

11.
Posterior reversible encephalopathy syndrome (PRES) associates various neurological manifestations (headaches, seizures, altered mental status, cortical blindness, focal neurological deficits, vomiting) and transient changes on neuroimaging consistent with cerebral edema. Posterior reversible encephalopathy syndrome mainly occurs in the setting of hypertension, eclampsia, renal failure and/or use of immunosuppressive drugs.We report four cases of PRES complicating systemic lupus erythematosus (SLE). In all our cases, renal involvement and hypertension were present. Neurological symptoms were typical. Magnetic resonance imaging showed posterior cerebral edema and in one case hemorrhagic complication. With symptomatic treatment and immunosuppressor withdrawal when they were previously used, symptoms fully resolved within 15 days in all cases, but one who had only partial regression related to cerebral hemorrhage.Including our cases, we reviewed a total of 46 patients with SLE and PRES. Their clinical and radiological presentation was not specific. The peculiar role of SLE itself in the occurrence of PRES was not clear, since hypertension (95%), renal involvement (91%), recent onset of immunosuppressive drugs (54%) and/or recent treatment with high intravenous dose of steroids (43%) were often present. The hypertension and other worsening factors should be treated. Finally, the evolution of this clinical and radiological spectacular syndrome is generally rapidly favorable.  相似文献   

12.
A variety of neuropsychiatric findings may complicate systemic lupus erythematosus (SLE) and pose diagnostic and therapeutic dilemmas. We describe the clinical and radiographic features of posterior reversible encephalopathy syndrome (PRES) and distinguish PRES from other conditions seen in SLE. Patient charts and magnetic resonance imaging (MRI) findings of four patients with SLE on immunosuppressive therapy with acute or subacute neurologic changes initially suggesting cerebritis or stroke were reviewed. The English language literature was reviewed using the Medline databases from 1996-2006 for other reports of PRES with SLE. Literature review yielded 26 other SLE cases reported with PRES. SLE patients with PRES were more commonly on immunosuppressive drugs, had episodes of relative hypertension, and had renal involvement. Characteristic findings are seen on MRI, which differentiate PRES from other CNS complications of SLE. Clinical and radiographic resolution of abnormalities within 1-4 weeks is typically seen. PRES has been increasingly recognized. Reversible changes are found on brain MRI accompanied by sometimes dramatic signs and symptoms. The therapeutic implications for separating PRES from stroke or cerebritis are important. We propose that PRES should be considered in the differential diagnosis in SLE patients with new-onset neurologic signs and symptoms.  相似文献   

13.
Posterior reversible encephalopathy syndrome (PRES) is a clinical syndrome that manifests with the onset of headache, confusion or decreased level of consciousness, visual changes, and seizures in conjunction with the typical neuroimaging features of posterior cerebral white matter changes, which are usually reversible. This syndrome has been associated with hypertension, eclampsia, renal insufficiency, immunosuppressive drugs, and connective-tissue diseases. To our knowledge, only four cases of PRES associated with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis have been reported in the literature. Here, we present a patient with ANCA-associated vasculitis complicated by PRES.  相似文献   

14.
OBJECTIVE: Posterior reversible encephalopathy syndrome (PRES) is a rare, recently described neurologic condition identifiable by clinical presentation and magnetic resonance image (MRI) appearance. It is associated with renal insufficiency, hypertension, and rheumatologic diseases. Patients present with headache, seizures, loss of vision and altered mental function, and a pattern on imaging studies of predominantly transient, posterior cerebral hyperintensities on T2-weighted MRI. There is a high likelihood of presentation of this syndrome to a rheumatologist. METHODS: Three recent cases of systemic lupus erythematosus (SLE) with PRES, along with 9 previously reported cases, are reviewed. RESULTS: All 3 patients presented with seizures and subacute visual changes in association with lupus nephritis. The first presented with hypertension, complete visual field loss, and status epilepticus 2 weeks after starting oral cyclosporine therapy for refractory lupus nephritis. The second patient was normotensive and presented with seizures and visual symptoms while in hospital with SLE-related pancreatitis and nephritis. The third patient had headache and seizures with severe lupus disease activity including nephritis, pancytopenia, and pulmonary hemorrhage. Cranial MRI showed predominantly posterior signal abnormalities on T2-weighted images, which resolved after cessation of cyclosporine in the first case, treatment with IV cyclophosphamide in the second case, and treatment with cyclophosphamide and plasmapheresis in the final case. Literature review showed that PRES is a manifestation of SLE or a consequence of therapy with calcineurin inhibitors or rituximab. The hallmark features are visual loss and seizures. Severe hypertension (> 170/110 mm Hg) and renal failure were present in the majority of previously identified cases of SLE and PRES. Our second case was normotensive but had marked lupus disease activity. PRES can lead to cerebral infarction. CONCLUSION: With increasing availability of MRI, PRES will be identified more frequently. Swift action to identify potential offending agents, controlling hypertension, and treating active disease can lead to reversal of radiologic and neurologic findings.  相似文献   

15.
Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic condition characterized by reversible vasogenic edema on neuroimaging. It is associated with various neurological manifestations, including headaches, vomiting, seizures, visual loss, altered mental status and focal neurological deficits. PRES mainly occurs in the setting of eclampsia, hypertension, uremia, malignancy, transplantation, autoimmune diseases and/or use of immunosuppressive drugs. This syndrome has been described in patients with systemic lupus erythematosus (SLE). PRES is a potentially reversible clinical-radiological entity; however, it can be complicated with vasculopathy, infarction or hemorrhage. Vasculopathy has been demonstrated to be a common finding in patients with SLE. We report the case of a woman with lupus nephritis and PRES whose diffuse vasculopathy was present on initial neuroimaging. Subsequent brain computed tomography scan demonstrated interval development of intraparenchymal hemorrhage and subarachnoid hemorrhage. To our knowledge, this unique brain image pattern has not been reported in SLE patients.  相似文献   

16.
Autoimmune vasculitides can have diverse neurological manifestations, including posterior reversible encephalopathy syndrome (PRES). Takayasu's arteritis (TA) is an uncommon vasculitis rarely associated with PRES. Common clinical features of TA include hypertension, audible arterial bruits, absence of peripheral pulses, claudication of the extremities, reduced blood pressure in one or both arms, and angiographic abnormalities. PRES has been mostly associated with severe hypertension, endothelial injury, and conditions such as renal disease, immunosuppressive medication use, and rheumatologic diseases. Headaches, seizures, and altered mental status are the main clinical features as well as characteristic findings in magnetic resonance imaging. TA frequently presents with hypertension and is associated with endothelial injury, making this entity an ideal setting for the development of PRES. We report the case of a 17-year-old female who presented to the emergency department with severe hypertension, headache, and seizures. Magnetic resonance imaging findings were suggestive of PRES. She had absent pulses in the right upper extremity, abdominal bruits, and angiographic findings included subclavian and renal artery stenoses. The diagnosis of TA was made, and she responded well to treatment. We found ten additional cases of TA and PRES in the literature. All patients were females under the age of 40, had renovascular hypertension, and presented with headaches and seizures. Current literature relevant to this rare association is presented and discussed.  相似文献   

17.
Posterior reversible encephalopathy syndrome (PRES) is a neurological disorder characterized by seizures, altered mental status and visual disorders, along with characteristic radiological findings. It is strongly related to hypertension induced by steroids and other immunosuppressive agents. There are an increasing number of reports regarding PRES arising during the course of chemotherapy for hematological malignancies. To clarify the risk factors for this phenomenon, we retrospectively analyzed pediatric patients undergoing treatment for hematological malignancies. Of 161 patients, six patients (3.7 %) developed PRES with characteristic clinical and radiographic findings. Univariate analysis revealed that tumor lysis syndrome (TLS) was a significant risk factor for the onset of PRES. TLS is a significant risk factor for the development of PRES in pediatric patients receiving chemotherapy for hematological malignancies.  相似文献   

18.
BACKGROUND: The development of central nervous system (CNS) symptoms in patients with preexisting systemic lupus erythematosus (SLE) evokes a wide differential diagnosis. Reversible posterior leukoencephalopathy (RPLE) is a rapidly evolving neurologic syndrome with characteristic clinical and radiographic features. Conditions commonly associated with RPLE include hypertensive encephalopathy, eclampsia, immunosuppressive drugs, and inflammatory disorders. OBJECTIVES: To describe our experience with RPLE in patients with concomitant SLE and review the literature. METHODS: The details of 5 novel cases and a MEDLINE review of the literature concerning the development of RPLE in association with SLE are presented. RESULTS: All cases included patients with SLE who developed the acute onset of headache, altered mental status, visual changes, and seizures. Neuroimaging demonstrated posterior white matter edema involving the parietal, temporal, and occipital lobes. Complete clinical and radiographic recovery occurred with prompt antihypertensive treatment and supportive care. Literature review identified 16 additional cases of RPLE occurring in patients with active SLE; the majority of these reports was similar in presentation and outcome to our experience. CONCLUSIONS: It is likely that the clinical manifestations and neuroimages in these lupus patients were the result of the RPLE syndrome. Fortunately, this cause of "secondary" CNS symptoms in patients with SLE is readily reversible when diagnosed early and treated with blood pressure control and supportive care.  相似文献   

19.
Rheumatologic diseases have varied clinical presentations, and posterior reversible encephalopathy syndrome (PRES) can be one of their presentations. The exact etiology of PRES is unknown, but endothelial dysfunction and immunosuppressive medications seem to be the likely cause in rheumatologic diseases. Clinical features include headaches, seizures, altered mental status, cortical blindness, vomiting, and focal neurologic deficits. The diagnosis of PRES can be difficult because several neuropsychiatric illnesses are generally prevalent in rheumatologic diseases; however, a high index of suspicion among physicians along with neuroimaging can help in the accurate diagnosis. Treatment guidelines are lacking, but in a few case series, lowering the blood pressure, controlling the seizures, and removing the immunosuppressive drugs have shown good results. There is need for randomized controlled trials addressing the treatment of PRES in rheumatologic diseases. Medline search was done from year 1950 to March 2011 using ??posterior reversible encephalopathy?? as keyword, and articles relevant to rheumatology were reviewed. We found 48 case reports showing PRES in patients with rheumatologic disease. Most of the patients were female. Age range was from 6 to 59?years. Out of the 48 case reports, 38 patients had systemic lupus erythematosus and most of them had renal disease. Five patients with autoimmune diseases presented with PRES after being started on immunomodulatory drugs. The most frequent symptoms were headache, seizures, and visual changes.  相似文献   

20.
To characterize reversible posterior leukoencephalopathy syndrome (RPLS) in systemic lupus erythematosus (SLE) in terms of treatments for resolution and its clinical course, we reviewed 28 cases of RPLS in SLE including our cases in view of the treatment. Of these, 15 cases improved with blood pressure control and 13 required immunosuppressive therapy for activity of SLE presenting neurological manifestations. Patients without immunosuppressants at onset of RPLS more frequently required immunosuppressive therapy to recover it than those precedingly using these agents [31% (4/13) versus 87% (13/15), p = 0.008, chi-square test]. Brain magnetic resonance imaging (MRI) is important for diagnosis of RPLS-SLE in the patient with SLE who develops neurological disturbance and rapidly increasing blood pressure. When 7-day therapy for hypertension and convulsion does not reverse the manifestations, immunosuppressive treatments would be recommended to reverse RPLS.  相似文献   

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