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.     

Infections in patients with systemic lupus erythematosus

Infection is a major contributor to morbidity and mortality in patients with systemic lupus erythematosus (SLE). In most clinical series, infection ranks first or second as the most common cause of death in SLE patients worldwide, including Hong Kong. In this article, the spectrum of infections and their protean manifestations in lupus patients will be reviewed with emphasis on clinical data from Hong Kong and other Asian countries. A high index of suspicion and dedicated work‐up to identify the causative pathogens is pivotal to the early diagnosis and effective management of infective complications in patients with SLE.     

系统性红斑狼疮脑病36例临床分析

目的研究系统性红斑狼疮脑病患者临床表现特点。方法分析、归纳2000-01-01~2004-10-10中日友好医院神经内科36例系统性红斑狼疮脑病患者症状、体征、辅助检查。结果36例系统性红斑狼疮脑病患者主诉症状9类,其中头痛、意识障碍、肢体无力居发生率前3位;发现体征13类,其中病理反射、意识障碍、肢体瘫痪居前3位。结论(1)系统性红斑狼疮脑病是活动性系统性红斑狼疮的表现之一。(2)系统性红斑狼疮患者无论病史长短,均可发生系统性红斑狼疮脑病。(3)系统性红斑狼疮脑病表现复杂多样。     

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.     

Recurrent fractures in an elderly patient with systemic lupus erythematosus

Glucocorticoid‐induced osteoporosis (GIO) is an important problem that remains undertreated, even by rheumatologists. We present a case of an elderly patient with systemic lupus erythematosus diagnosed more than 40 years ago, who suffered from recurrent fractures and attendant complications despite a bone mineral density (BMD) score in the osteopenic range and treatment with bisphosphonates. With improved treatment and outcome of lupus, an increasing number of elderly patients who are susceptible to osteoporotic fractures are expected. This case serves to highlight that rheumatic disease patients on steroids should be screened for GIO, as effective treatment and preventive measures are available. Teriparatide is a promising treatment for patients who have failed bisphosphonate treatment or who are at high risk for fracture. We should also bear in mind that BMD scores alone are not indicative of fracture risk, and other tools such as the WHO‐FRAX (Fracture risk assessment tool), serum vitamin D3 levels and bone turnover markers should be used where appropriate. Other measures including attention to factors that contribute to falls should also be considered, necessitating a multi‐disciplinary approach.     

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.     

Pulmonary diffusion capacity in patients with systemic lupus erythematosus

OBJECTIVE: The aim of this study was to clarify the characteristics of pulmonary function tests (PFT), especially carbon monoxide diffusion capacity (DLCO), and their correlation with clinical features and immunological findings in patients with systemic lupus erythematosus (SLE). METHODOLOGY: Vital capacity (VC) and DLCO were analysed retrospectively in 110 sequential Japanese SLE patients with active disease between 1985 and 1999. In 38 patients, serial measurements of PFT were also assessed during high-dose corticosteroid therapy. RESULTS: DLCO was reduced in 52 patients (47%) and a restrictive impairment of PFT was observed in nine patients (8%). The prevalence of pulmonary fibrosis was 13%. Reduced DLCO was frequently observed, even in patients with neither pulmonary fibrosis nor a restrictive pattern. No correlation between immunological data and reduced DLCO was found, except for the presence of anti-RNP. Patients with Raynaud's phenomenon showed a higher prevalence of DLCO impairment than those without this phenomenon. Although immunological parameters improved significantly after the corticosteroid therapy, no significant change in the level of DLCO was observed. CONCLUSIONS: Impairment of DLCO was frequently observed in patients with SLE who had no clinical respiratory abnormalities. DLCO impairments were correlated with Raynaud's phenomenon clinically, and the presence of anti-RNP immunologically. No significant correlation was found between impairment of DLCO and disease activity of SLE.     

Measuring functional status in patients with systemic lupus erythematosus

With improvements in mortality in systemic lupus erythematosus (SLE), the functional status of these patients, assessed using health‐related quality‐of‐life (HRQoL) measures, is increasingly being recognized as an important outcome measure in clinical research. Domains of HRQoL of particular importance to SLE patients include fatigue, ability to work, good health, independence and social and family life. The SF‐36 currently appears to be the best available measure for assessment of HRQoL in SLE. It measures some of the domains of importance to SLE patients, has good psychometric properties, patient acceptability, construct validity and has been validated for use in many languages. Using HRQoL measures, it has been shown that SLE patients have poorer functional status than the general population, and that specific manifestations of SLE (e.g. fibromyalgia and renal failure) may influence HRQoL in these patients. Prospective and cross‐sectional studies have identified a variety of factors – some potentially modifiable – which influence HRQoL in SLE. Additional research is needed to identify strategies which can improve HRQoL in SLE patients.     

Risk factors for avascular necrosis among Filipino patients with systemic lupus erythematosus

Aim: To describe the clinical features and risk factors for avascular necrosis (AVN) in a cohort of Filipino patients with systemic lupus erythematosus (SLE). Methods: We reviewed the medical records of SLE patients with a diagnosis of AVN, seen at the University of Santo Tomas (Manila, Philippines) Section of Rheumatology, from 1995 to 2005. The diagnosis of AVN was based on clinical symptoms and confirmed by plain radiographs or magnetic resonance imaging. Possible risk factors for the development of AVN were identified. The clinical data of SLE patients without AVN were also obtained and served as controls. Results: Of the 540 patient charts reviewed, 43 (8.0%) patients (41 female, 2 male) with AVN were included. Out of a total of 66 joints involved, the hip was the most frequently involved. We included 93 SLE patients without AVN who were matched for age, sex and disease duration as the control group. Mean daily prednisone dose (11.9 ± 7.2 vs 9.3 ± 6.6 mg, P = 0.023), mean cumulative prednisone‐equivalent dose in first month of SLE diagnosis (1.5 ± 0.8 vs 1.3 ± 0.8 g, P = 0.011), and total cumulative prednisone‐equivalent dose (30.0 ± 2.7 vs 20.3 ± 1.9 g, P = 0.023) were higher in the AVN group than in the controls. Clinical variables significantly associated with AVN included the presence of vasculitis (OR = 4.45, 95% CI 1.65–12.18, P = 0.0007), the use of intravenous pulse steroids (OR = 2.92, 95% CI 1.21–7.08, P = 0.008), and the mean total cumulative prednisone‐equivalent dose ≥ 23.4 g (OR = 2.92, 95% CI 1.3–6.6, P = 0.007). Conclusion: Corticosteroid use and vasculitis were consistent risk factors seen among Filipino SLE patients who developed AVN during the course of their disease.     

Central nervous system infections in Filipino patients with systemic lupus erythematosus

Background and purpose: Infections including those of the central nervous system (CNS) are a major contributor to morbidity and mortality in systemic lupus erythematosus (SLE). This case series describes the etiology, contributing factors and outcomes of CNS infections in a group of Filipino patients with SLE. Design: Retrospective case series. Methods: We reviewed the medical records of SLE patients diagnosed and confined for a CNS infection at the University of Santo Tomas Hospital in Manila, Philippines, from 1997 to 2007. Results: A total of 23 SLE patients (22 females) diagnosed with CNS infection were included in this study. The mean age was 25.8 years (range 12–51) at SLE diagnosis, and 30.9 years (range 14–58) at CNS infection, with a mean disease duration of 55 months (range 7–125). Nineteen cases (82.6%) were meningitis, and four (17.4%) were diagnoses of brain abscess. The etiologic agents were identified as Cryptococcus neoformans in seven (30.4%), Mycobacterium tuberculosis in seven (30.4%), Streptococcus pneumoniae in two (8.7%), Salmonella sp. in one (4.4%), Corynebacterium bovis with Actinomyces sp. in one (4.4%), and no isolate in five (21.7%). The average daily prednisone dose was 28.9 mg (range 0–60 mg); 10 patients had recently received pulse cyclophosphamide, and two were on mycophenolate mofetil at the time of infection. Most cases had active SLE; the lone patient in disease remission had S. pneumoniae meningitis post‐splenectomy. The most common presentation was headache (100%) and fever (87%). The infection resolved completely in nine patients (39.1%), and resolved with sequelae in two patients (8.7%); 12 patients (52.2%) died. Conclusion: We described the etiology and outcomes of CNS infections in a group of Filipino patients with SLE. Risk factors included active SLE in the majority of cases requiring moderate‐ to high‐dose steroids and other immunosuppressants like cyclophosphamide. Although C. neoformans and M. tuberculosis were the most common etiologic agents, it is just as important to search for less common organisms which can produce disease in highly susceptible hosts. A high index of suspicion and early appropriate management are crucial to favorable outcome among these patients.     

Salmonella infections in patients with systemic lupus erythematosus: a case series

Background: Systemic lupus erythematosis (SLE) patients are highly susceptible to infections. Aim: We aim to present our experience regarding the manifestations and outcomes of Salmonella infections in a group of Filipino SLE patients. Methods: This study reviewed the medical records of Filipino SLE patients with documented Salmonella infection seen at the Rheumatology Clinics of the University of Santo Tomas (UST) Hospital, Manila, Philippines from 2002 to 2009. Results: Included were 12 patients (11 female), with mean age of 25.75 years (range 11–53 years) and average SLE disease duration 4.45 years to Salmonella infection. Five had septic arthritis (knee in 4, hip in 1), one of whom had concomitant typhoid fever. Another two patients had typhoid fever only. Sepsis syndrome was noted in three. One patient each had soft tissue abscess and meningitis. Salmonella typhi was isolated in seven patients, Salmonella sp. in four, and S. enteritidis in one patient. Two patients developed acute respiratory distress syndrome (ARDS), another had mixed Salmonella and Klebsiella pneumonia. Eleven patients were on high‐dose steroids for active disease, including one patient with antecedent first infusion of cyclophosphamide plus rituximab. Management consisted of antimicrobials and adjunct surgery for arthritis and abscess. Except for three patients with Salmonella sepsis, all other patients improved with appropriate therapy.     

Chronic intestinal pseudo-obstruction in systemic lupus erythematosus

Background/Aims—Chronicintestinal pseudo-obstruction (CIPO) reflects a dysfunction of thevisceral smooth muscle or the enteric nervous system. Gastrointestinalmanifestations are common in systemic lupus erythematosus (SLE)but CIPO has not been reported. Features of CIPO are reported in fivepatients with SLE.
Methods—From 1988 to1993, five patients with SLE or SLE-like syndrome were hospitalised forgastrointestinal manometric studies. CIPO was the onset feature in twocases. Antroduodenal manometry (three hours fasting, two hours fed) wasperformed in all patients, and oesophageal manometry in four.
Results—Intestinalhypomotility associated with reduced bladder capacity and bilateralureteral distension was found in four patients and aperistalsis of theoesophagus in three. Treatment, which consisted of high dosecorticosteroids, parenteral nutrition, promotility agents, andantibiotics, led to remission of both CIPO and urinary abnormalities inall cases. Antroduodenal manometry performed in two patients afterremission showed increased intestinal motility. One patient died, andpostmortem examination showed intestinal vasculitis.
Conclusions—CIPO inSLE is a life threatening situation that can be reversed by treatment.It may be: (a) a complication or onsetfeature of the disease; (b) secondary tosmooth muscle involvement; (c) associatedwith ureteral and vesical involvement; (d)the result of intestinal vasculitis.

Keywords:chronic intestinal pseudo-obstruction; systemiclupus erythematosus

     

165例系统性红斑狼疮患者血液系统受累情况分析

目的 探讨系统性红斑狼疮(SLE)患者血液系统受累特点,为其诊治提供依据.方法 对165例确诊的SLE患者血液系统受累情况进行回顾性分析.结果 本组96例有贫血,其中慢性疾病性贫血(ACD) 63例,自身免疫性溶血性贫血(AIHA) 11例;72例外周血白细胞下降,65例血小板下降.49例行骨髓检查,以增生活跃为主(44例).结论 SLE血液系统受累发生率高、范围广.对血象异常的育龄女性患者应注意进行SLE的相关筛查.对于诊断为AIHA或特发性血小板减少性紫癜的患者,应警惕同时存在SLE的可能性.     

Non‐aneurysmal subarachnoid hemorrhage in two patients with systemic lupus erythematosus: Case reports and literature review

We describe two cases of non‐aneurysmal subarachnoid hemorrhage (SAH) and multifocal stenosis of the intracranial arteries. The patients' histories together with magnetic resonance angiography, vessel wall imaging and transcranial Doppler (TCD) indicated that the SAH was due to vasculitis or reversible cerebral vasoconstriction syndrome (RCVS). Differential diagnosis of vasculitis and RCVS is important because the treatment strategies are different: immunosuppressants in vasculitis and calcium channel blockers in RCVS. Vessel wall magnetic resonance imaging and TCD can be helpful in differentiating them.