Clinical features of reversible posterior leukoencephalopathy syndrome in patients with systemic lupus erythematosus

Abstract

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.     

系统性红斑狼疮合并可逆性脑白质后部综合征1例报道

目的 探讨系统性红斑狼疮（SLE）合并可逆性脑后部白质综合征（RPLS）的诱发因素、临床特点及诊治要点,以提高临床医生对该病的认识,降低误诊率。方法 对近期我科收治的1例SLE合并RPLS的临床资料进行了深入分析,并复习相关文献。结果 患者原发病诊断明确,入院后双手颤抖伴癫痫失神发作,予以大剂量激素联合他克莫司治疗及抗癫痫处理后效果不佳,停用他克莫司及积极控制血压等处理后,未再出现类似症状,头颅MRI提示病变范围较前明显缩小,综合考虑诊断为SLE合并RPLS。结论 SLE合并RPLS极易与神经精神狼疮、高血压脑病等疾病混淆,临床应提高对该病的认识和警惕,避免误诊误治。     

Reversible posterior leukoencephalopathy in patients with systemic lupus erythematosus

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.     

系统性红斑狼疮合并脑后部可逆性脑病综合征的临床分析

目的 脑后部可逆性脑病综合征(PRES)是一种有特征性临床和影像学改变的神经精神综合征.通过分析系统性红斑狼疮(SLE)合并PRES患者的临床表现和复习文献,探讨其发病特点.方法 分析4例确诊为SLE合并PRES患者的临床特点,收集其临床表现、发病诱因和影像学检查资料.结果 4例和文献报道共48例SLE合并PRES患者,发生高血压为42例(88%),血肌酐升高为30例(63%),39例使用激素、免疫抑制剂或细胞毒药物(81%).头痛46例(96%),视觉障碍28例(58%),意识障碍20例(42%),癫痫发作43例(90%),CT及磁共振成像(MRI)显示多发病灶主要位于双侧枕叶、顶叶、额叶、颞叶和小脑.降压和及时处理诱因症状可缓解.结论 PRES是一种临床-影像综合征,常与高血压、肾功能衰竭、免疫抑制剂等药物相关,SLE合并PRES若能快速诊断并给予正确治疗可使症状缓解,病变可逆.     

Clinical features of patients with mild systemic lupus erythematosus.

Clinical features of 16 patients with mild systemic lupus erythematosus (SLE) were compared with those of 21 control patients with moderate or severe disease. Age at the time of diagnosis of SLE was higher in mild disease group. The incidence of the coexistence of Sj?gren's syndrome (SS) at the time of diagnosis of SLE was higher in patients who later developed mild disease. Anti-Sm antibody and decreased levels of C3, C4, and CH50 occurred less frequently in patients with mild disease. SLE patients with the coexistence of SS at the time of diagnosis of SLE may represent a subset with a benign prognosis.     

Clinical features of systemic lupus erythematosus

We compared the frequency of clinical features of systemic lupus erythematosus (SLE) and determined survival in 113 patients with younger-onset lupus (age ⩾ < at clinical diagnosis) and 25 patients with older-onset disease (age ⩾55 at diagnosis). The most striking difference was in the racial distribution; 59% of the younger patients were black, compared with only 20% of the older-onset patients (P < 0.001). Major manifestations of lupus (including clinically evident renal disease, central nervous system involvement, cutaneous involvement, and hemocytopenia) occurred with similar frequency in both age groups. Antibodies to DNA were detectable equally often in both groups, but hypocomplementemia was more common in the younger patients. Five-year survival in the younger-onset group (79%) was similar to that of the older-onset group (72%); there was a tendency toward relatively improved survival in patients in the latter group when compared with the expected survival of appropriately matched control populations. Major significant differences in racial distribution included 1) a higher incidence of serositis in older whites and in blacks regardless of age, and 2) more frequent hypocomplementemia in younger patients within both racial groups.     

老年人可逆性后部白质脑病综合征五例及临床和影像学特点分析

目的 分析我国老年人可逆性后部白质脑病综合征(reversible posterior leukoencephalopathy syndrome,RPLS)患者的临床表现和影像学特点.方法 回顾报告5例老年RPLS患者的临床表现和影像学特点. 结果 本组老年男性患者1例,女性4例,平均年龄(63.0±2.4)岁,5例患者均在起病时并存高血压.最常见的临床表现依次为视觉障碍5例,头痛4例,癫痫4例,意识障碍3例,病理反射2例,偏瘫2例.CT检查示4例患者表现异常,MRI检查示5例患者均出现枕叶病变,颞叶受累2例,顶叶受累1例.4例患者出院平均(48.8±29.2)d后CT检查为阴性结果,死亡1例.结论 RPLS是典型的多因共果疾病,血管原性假说被影像学研究结果证实.神经影像学对诊断RPLS很重要,弥散加权像(DWI)有助于鉴别诊断RPLS和缺血性脑血管病.     

Clinical features,outcome, and associated factors for posterior reversible encephalopathy in Thai patients with systemic lupus erythematosus: a case-control study

Posterior reversible encephalopathy syndrome (PRES) in patients with systemic lupus erythematosus (SLE) has been recognized increasingly. This study aimed to determine the prevalence, clinical features, brain imaging findings, outcomes, and associated factors of PRES in Thai SLE patients. SLE patients with PRES were identified from the lupus cohort of Chiang Mai University. Controls were SLE patients with a hospital number close to and actually had SLE diagnosis within 5 years of the case (case:control ratio = 1:4). Of 1,332 SLE patients, 30 episodes of PRES were identified in 24 female SLE patients (prevalence 1.80%). The mean ± SD age at SLE diagnosis and at onset of PRES was 25.02 ± 13.78 and 28.31 ± 12.61 years, respectively. Seizure was the most common presenting symptom, as seen in 28 episodes, followed by acute severe headache in 17, alteration of consciousness in 17, nausea and vomiting in 10, blurred vision in 11, and hemiparesis in 3. Abrupt increase in blood pressure and active nephritis were seen in 29 and 26 of the episodes, respectively. Urine protein/creatinine ratio > 1.00 (OR 15.72, 95% CI 3.12–79.12, p = 0.001) and hemoglobin < 10 gm/dL (OR 5.12, 95% CI 1.37–19.15, p = 0.015) were associated factors for developing PRES. During the observation period, 7 patients in the PRES group and 8 in the control group died (p = 0.015). PRES was uncommon in SLE patients, but associated with a high mortality rate. Active nephritis and anemia were associated factors of PRES in Thai SLE patients.     

Clinical features of Pneumocystis pneumonia in patients with systemic lupus erythematosus]

Systemic lupus erythematosus (SLE) is often associated with various opportunistic infections, particularly during treatment with corticosteroids or immunosuppressants. We studied the clinical characteristics of 15 patients with SLE who underwent diagnostic bronchoalveolar lavage (BAL) and compared 6 patients with confirmed Pneumocystis pneumonia (PcP+), with 9 patients without Pneumocystis pneumonia (PcP-). The serum concentrations of beta-D-glucan and KL-6 were significantly higher in PcP+ than in PcP- patients, whereas serum LDH was similar in both groups. The serum concentrations of complement, a marker of SLE activity, and of IgG did not predict the presence of PcP. In all patients, the overall cell and lymphocyte counts were increased in the BAL fluid, without any significant difference between the PcP+ and PcP- groups. Ground-glass opacities on chest computed tomography, and oxygenation impairment (PaO2/FiO2<200Torr) were more common in PcP+ than PcP- patients. We concluded that, in patients with SLE, serum beta-D-glucan and KL-6 might be useful in the diagnosis of PcP, particularly when severe hypoxemia precludes BAL.     

可逆性后部白质脑病综合征的发病机制

可逆性后部白质脑病综合征(reversible posterior leukoencephalopathy syndrome,RPLS)是一种临床影像学疾病实体,主要以迅速进展的血压增高、头痛、呕吐、意识障碍、痢性发作为特征,神经影像学显示为双侧大脑半球对称性白质可逆性水肿(尤其是在大脑后部),通过及时和正确的治疗,临床症状和神经影像学改变可完全恢复.有关RPLS发病机制的两大假说--脑血管痉挛学说和脑血管过度灌注学说一直存在争论,目前多数学者仍然认同后者是造成脑水肿的主要原因.文章从RPLS的病因学、病理学和影像学特征阐述了其发病机制以及近年来对上述两大假说的新观点.     

Reversible posterior encephalopathy syndrome in systemic lupus erythematosus and lupus nephritis

Reversible posterior encephalopathy syndrome (RPES) is a clinical entity characterized with headache, nausea, vomiting, seizures, consciousness disturbance, and frequently visual disorders associated with neuroradiological findings, predominantly white matter abnormalities of the parieto-occipital lobes. The central nervous system manifestations of systemic lupus erythematosus (SLE) are highly diverse. However, SLE-associated RPES has been seldom reported. Here, we report a case with RPES in SLE and lupus nephritis with exclusive involvement of parietal and occipital cortices. A systematic review of the literature on the pathogenesis and treatment of SLE-associated RPES is included.     

Progressive multifocal leukoencephalopathy restricted to the posterior fossa in a patient with systemic lupus erythematosus

Progressive multifocal leukoencephalopathy is a neurological infectious disease caused by the John Cunningham polyomavirus (JCV), an opportunistic agent with worldwide distribution. This disease is frequently seen in immunosuppressed patients and rarely associated with systemic lupus erythematosus. In the central nervous system PML demyelinating lesions occur in the supratentorial compartment. The authors describe a rare case of PML secondary to SLE treatment with atypical presentation restricted to the posterior fossa.     

可逆性大脑后部白质脑病综合征16例的临床分析

目的探讨可逆性大脑后部白质脑病综合征的病因和临床特点,寻找早期诊断和治疗方法。方法选取该院2010．02～2013-02收治的16例可逆性大脑后部白质脑病综合征患者,回顾性分析其病因、临床表现特点、治疗及预后。结果16例患者中男6例,女10例。病因：高血压脑病7例（其中合并肾功能不全4例）,子痫4例,多发性大动脉炎2例,血栓性血小板减少性紫癜1例,系统性红斑狼疮1例,右颈内动脉内膜剥脱术后1例。临床表现以头痛、谵妄、意识障碍、癫痫发作性抽搐、视幻觉为主。头颅MRI检查可见大脑半球后部白质为主的T1加权呈短信号及12加权呈长信号的病灶,常双侧对称。经及时适当的脱水、降颅压,改善循环和高压氧等治疗,全组16例中,症状与体征消失者14例（87．5％）,明显好转者2例（12．5％）。结论可逆性大脑后部白质脑病综合征是一种具有自身病因、临床表现特点的临床综合征,经及时有效治疗,大多预后较好。     

系统性红斑狼疮患者结核菌感染的临床特点及相关因素分析

目的分析系统性红斑狼疮（SLE）患者结核菌感染的临床表现、特点以及相关危险因素。方法回顾分析了37例SLE结核感染住院患者和65例随机抽取的SLE对照组患者的临床资料．所有患者均有完整的病史、体格检查、实验室等辅助检查的资料。结果在37例SLE结核感染患者中．肺结核13例（35％），肺结核合并其他部位结核者7例，肺外结核17例。与对照组相比，结核感染组的日平均激素的剂量和细胞毒药物使用较高，存两组之间差异有统计学意义，而性别、年龄、病程、白蛋白／球蛋白、补体和激素冲击治疗等方面，两组问差异无统计学意义。讨论SLE患者发生肺外结核感染的机会较大，且日平均激素的剂量和细胞毒药物是SLE患者发生结核感染的危险因素。     

Systemic lupus erythematosus complicated with posterior reversible encephalopathy syndrome and intracranial vasculopathy

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.