Neuropsychiatric lupus in late- and early-onset systemic lupus erythematosus patients: a systematic review and meta-analysis.

OBJECTIVES: Late-onset SLE is usually milder and associated with lower frequency of LN and neuropsychiatric manifestations. The diagnosis of NPSLE is especially challenging in older patients because of increased incidence of neurological comorbidities. We performed a systematic review and meta-analysis to evaluate the differences in NPSLE manifestations in early-onset (<50-year-old) vs late-onset (≥50-year-old) SLE patients. METHODS: A literature search was performed using the PubMed, Web of Science and Cochrane Library databases. Studies available in English (1959-2022) including a late-onset SLE comparison group and evaluating the frequency of NPSLE were eligible. A forest plot was used to compare odds ratios (95% CI) of incidence and manifestations of NPSLE by age groups. Study heterogeneity was assessed using I2 statistics. RESULTS: A total of 44 studies, including 17 865 early-onset and 2970 late-onset SLE patients, fulfilled our eligibility criteria. CNS involvement was reported in 3326 patients. Cumulative NPSLE frequency was higher in the early-onset group than in the late-onset group (OR: 1.41, 95% CI: 1.24, 1.59, P < 0.0001). In early-onset SLE patients, seizures (OR: 1.68, 95% CI: 1.27, 2.22) and psychosis (OR: 1.72, 95% CI: 1.23, 2.41) were more common than in late-onset SLE patients (P values, 0.0003 and 0.0014, respectively). Peripheral neuropathy was more commonly reported in the late-onset SLE group than in the early-onset SLE group (OR: 0.64, 95% CI: 0.47, 0.86, P = 0.004). CONCLUSION: Our meta-analysis revealed that the frequencies of overall NPSLE, seizures, and psychosis were less common in late-onset SLE patients than in early-onset SLE patients. In contrast, peripheral neuropathy was more common in the late-onset SLE group.

Central nervous system involvement in systemic lupus erythematosus: Data from the Spanish Society of Rheumatology Lupus Register (RELESSER).

OBJECTIVES: To analyze the prevalence, incidence, survival and contribution on mortality of major central nervous system (CNS) involvement in systemic lupus erythematosus (SLE). METHODS: Patients fulfilling the SLE 1997 ACR classification criteria from the multicentre, retrospective RELESSER-TRANS (Spanish Society of Rheumatology Lupus Register) were included. Prevalence, incidence and survival rates of major CNS neuropsychiatric (NP)-SLE as a group and the individual NP manifestations cerebrovascular disease (CVD), seizure, psychosis, organic brain syndrome and transverse myelitis were calculated. Furthermore, the contribution of these manifestations on mortality was analysed in Cox regression models adjusted for confounders. RESULTS: A total of 3591 SLE patients were included. Of them, 412 (11.5%) developed a total of 522 major CNS NP-SLE manifestations. 61 patients (12%) with major CNS NP-SLE died. The annual mortality rate for patients with and without ever major CNS NP-SLE was 10.8% vs 3.8%, respectively. Individually, CVD (14%) and organic brain syndrome (15.5%) showed the highest mortality rates. The 10% mortality rate for patients with and without ever major CNS NP-SLE was reached after 12.3 vs 22.8 years, respectively. CVD (9.8 years) and organic brain syndrome (7.1 years) reached the 10% mortality rate earlier than other major CNS NP-SLE manifestations. Major CNS NP-SLE (HR 1.85, 1.29-2.67) and more specifically CVD (HR 2.17, 1.41-3.33) and organic brain syndrome (HR 2.11, 1.19-3.74) accounted as independent prognostic factors for poor survival. CONCLUSION: The presentation of major CNS NP-SLE during the disease course contributes to a higher mortality, which may differ depending on the individual NP manifestation. CVD and organic brain syndrome are associated with the highest mortality rates.

[Neuropsychiatric manifestations in patients with systemic lupus erythematosus: about 108 cases in Cameroon]. / Manifestations neuropsychiatriques au cours du lupus érythémateux systémique: à propos de 108 cas au Cameroun.

Few data on neuropsychiatric disorders in systemic lupus erythematosus (NPSLE) are available in sub-Saharan Africa. The purpose of this study was to determine their frequency and describe their features in Cameroon. We conducted a retrospective study collecting all the medical records of patients with systemic lupus erythematosus (SLE) hospitalized in the department of rheumatology of 3 hospitals in Cameroon from 2009 to 2019. Lupus activity was assessed using the SLE activity index (SLEDAI). A total of 108 records of patients with a mean age of 40.2 ± 13.7 years were included in the study. The frequency of NPSLE was 55.5% (n=60). Neuropsychiatric disorders were diagnosed concomitantly with SLE (37.0%; n = 40) while in 20 patients with SLE (18.5%) they occurred during the first year. When NPSLE were inaugural, central nervous system involvement was dominant, with demyelinating syndrome 27.8% (n=30) and headaches 21.3% (n=23). Mononeuropathy was the most frequent peripheral nervous system involvement (15.7%; n=17). Factors associated with NPSLE occurrence were malar rash (p=0.024), alopecia (p=0.024), very high lupus activity (p=0.011), arthralgia (p<0.001), anti-nuclear factor (p=0.002). NPSLE did not appear to influence either lupus activity (log rank p=0.227) or the probability of new onset lupus (log rank p=0.233). More than half of patients had NPSLE during the first year. The presence of cutaneous and articular signs, high lupus activity, and anti-nuclear factor were associated with the occurrence of NPSLE.

Prevalence of Neuropsychiatric Lupus in Psychosis Patients Who Have Tested Positive for Antinuclear Antibodies.

OBJECTIVE: Psychosis is a rare manifestation of neuropsychiatric systemic lupus erythematosus (NPSLE). Current guidelines do not make a recommendation regarding the use of antinuclear antibody (ANA) testing in the assessment of patients with psychosis. The present study was undertaken to determine the prevalence of NPSLE in patients with psychosis who were positive for ANAs. METHODS: A retrospective review of patients who were admitted to the mental health service of 2 metropolitan tertiary referral centers with a diagnosis of psychosis and had been tested for ANAs was conducted. A diagnosis of SLE was made when the 2019 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria were fulfilled. Attribution of psychosis-related events to NPSLE were made according to validated criteria. RESULTS: There were 10,205 mental health admissions with diagnoses of psychosis representing 4,766 individual patients, 911 patients (19%) were tested for ANAs, 135 (15%) of those tests returned a positive result with a titer of ≥1:160. The mean ± SD follow-up time was 47 ± 26 months. At discharge, there were 4 patients who met 2019 ACR/EULAR criteria for SLE, 2 of whom met criteria for NPSLE (2 patients had other manifestations of SLE), yielding an NPSLE prevalence of 1.5% (2 of 135) among patients who were positive for ANAs, and 0.2% (2 of 911) among all patients who underwent testing for ANAs. CONCLUSION: The prevalence of NPSLE in patients with psychosis who were positive for ANAs was low, at 1.5%. The low rate of clinically significant positive results would argue against routine testing for ANAs in patients with psychosis.

Neuropsychiatric Systemic Lupus Erythematosus in Older Adults: Diagnosis and Management.

Systemic lupus erythematosus (SLE) is a multisystem chronic autoimmune disease with variable clinical manifestations. Neuropsychiatric systemic lupus erythematosus (NPSLE) includes the neurologic syndromes of the central, peripheral and autonomic nervous system and the psychiatric syndromes observed in patients with SLE. Neuropsychiatric systemic lupus erythematosus events may present as an initial manifestation of SLE or may be diagnosed later in the course of the disease. Older adults with NPLSE include those who are ageing with known SLE and those with late-onset SLE. The diagnosis of NPSLE across the lifespan continues to be hampered by the lack of sensitive and specific laboratory and imaging biomarkers. In this review, we discuss the particular complexity of NPSLE diagnosis and management in older adults. We first discuss the epidemiology of late-onset NPSLE, then review principles of diagnosis of NPSLE, highlighting issues that are pertinent to older adults and that make diagnosis and attribution more challenging, such as atypical disease presentation, higher medical comorbidity, and differences in neuroimaging and autoantibody investigations. We also discuss clinical issues that are of particular relevance to older adults that have a high degree of overlap with SLE, including drug-induced lupus, cerebrovascular disease and neurocognitive disorders. Finally, we review the management of NPSLE, mainly moderate to high- dose glucocorticoids and immunosuppressants, again highlighting considerations for older adults, such as increased medication (especially glucocorticoids) adverse effects, ageing-related pharmacokinetic changes that can affect SLE medication management, medication dosing and attention to medical comorbidities affecting brain health.

Neuropsychiatric involvement in juvenile-onset systemic lupus erythematosus: Data from the UK Juvenile-onset systemic lupus erythematosus cohort study.

INTRODUCTION: Juvenile-onset systemic lupus erythematosus (JSLE) is a rare autoimmune/inflammatory disease with significant morbidity and mortality. Neuropsychiatric (NP) involvement is a severe complication, encompassing a heterogeneous range of neurological and psychiatric manifestations. METHODS: Demographic, clinical, and laboratory features of NP-SLE were assessed in participants of the UK JSLE Cohort Study, and compared to patients in the same cohort without NP manifestations. RESULTS: A total of 428 JSLE patients were included in this study, 25% of which exhibited NP features, half of them at first visit. Most common neurological symptoms among NP-JSLE patients included headaches (78.5%), mood disorders (48.6%), cognitive impairment (42%), anxiety (23.3%), seizures (19.6%), movement disorders (17.7%), and cerebrovascular disease (14.9%). Peripheral nervous system involvement was recorded in 7% of NP-SLE patients. NP-JSLE patients more frequently exhibited thrombocytopenia (<100 × 109/L) (p = 0.04), higher C-reactive protein levels (p = 0.01), higher global pBILAG score at first visit (p < 0.001), and higher SLICC damage index score at first (p = 0.02) and last (p < 0.001) visit when compared to JSLE patients without NP involvement. CONCLUSIONS: A significant proportion of JSLE patients experience NP involvement (25%). Juvenile-onset NP-SLE most commonly affects the CNS and is associated with increased overall disease activity and damage.

Neuro-psychiatric manifestations in patients with systemic lupus erythematosus: A systematic review and results from the Swiss lupus cohort study.

OBJECTIVES: Systemic lupus erythematosus (SLE) is a systemic autoimmune disease associated with neuro-psychiatric (NP) manifestations. Frequency and patterns of neuro-psychiatric systemic lupus erythematosus (NPSLE) vary substantially between patients. We conducted a systematic review (SR) of the literature and examined prevalence and characteristics of NPSLE in the Swiss SLE cohort study (SSCS). METHODS: The SR search was performed between January 1999 and January 2020. We included prospective/cross-sectional studies focusing on NPSLE. We secured study characteristics, cohort compositions and frequencies of NP manifestations, assessed heterogeneity across reports and investigated sources of variation using meta-regression models. Regarding the SSCS, we reviewed all patients included and classified NP manifestations. RESULTS: The SR searches identified 530 studies. We included 22 studies in our meta-analysis, the mean frequency of NPSLE ranged from 10.6% to 96.4%. The frequency of NPSLE in the SSCS was 28.1%. Severe events including cerebrovascular insults, seizures and psychosis appeared in 7.1%, 5.3% and 6.5% respectively. There was a linear relationship between duration of SLE and cumulative incidence of NPSLE. CONCLUSIONS: The spectrum of NPSLE is very broad. The diagnostic work-up and rates of reported manifestations varied substantially across studies. We call for concerted efforts and consensus regarding definitions of NPSLE that will facilitate accurate diagnosis and attribution to SLE, particularly with a view to timely intervention and patient outcomes.

Factors associated with neuropsychiatric involvement in Latin American patients with systemic lupus erythematosus.

INTRODUCTION: Factors related to presentation of neuropsychiatric (NP) SLE manifestations, early in the course of the disease, and during follow up have not been clearly established. PURPOSE: To identify disease and non-disease related factors associated with NP manifestations in early SLE. METHODS: We included 1193 patients from the GLADEL inception cohort free of NP involvement at cohort entry. We evaluated the association of demographic, clinical and laboratory data with NP involvement during follow-up. STATISTICAL METHODS: Independent factors associated with NP involvement were identified using a multivariable Cox regression model. RESULTS: Factors independently associated with NP manifestations were: mestizo ethnicity (HR 1.701, 95% CI 1.282-2.258, p = 0.0002), myalgias/myositis (HR 1.832, 95% CI 1.335-2.515, p = 0.0002), pneumonitis (HR 2.476, 95% CI 1.085-5.648, p = 0.0312), shrinking lung (HR 2.428, 95% CI 1.074-5.493, p = 0.0331) and hemolytic anemia (HR 1.629, 95% CI 1.130-2.347, p = 0.0089). Longer disease duration at cohort entry (13 to 24 months) was associated with a lower risk of developing NP manifestations (HR 0.642, 95% CI 0.441-0.934, p = 0.0206). CONCLUSIONS: Patients with myalgias/myositis, pneumonitis, shrinking lung and hemolytic anemia are at higher risk of NP involvement, whereas longer disease duration at cohort entry is associated with a lower risk of developing NP involvement.

Clinical Features and Outcomes of Neuropsychiatric Systemic Lupus Erythematosus in China.

OBJECTIVE: To identify the clinical characteristics, magnetic resonance imaging (MRI) results, and prognostic factors of neuropsychiatric (NP) systemic lupus erythematosus (SLE; NPSLE) in a relatively large patient series in China. METHODS: Data of patients with NPSLE at Peking Union Medical College Hospital (PUMCH) were collected retrospectively from June 2012 to June 2016. NPSLE patients were compared with 220 non-NPSLE patients. Survival rates were evaluated using the Kaplan-Meier curves, log-rank test, and Cox proportional hazards modeling. Cranial MRI results were also studied. RESULTS: Of the 194 included patients, sixteen subtypes of NPSLE were identified, and the most common manifestations were seizure (36.6%), acute confusional state (25.3%), and cerebral vascular disease (15.5%). Compared with the non-NPSLE group, NPSLE patients were significantly more likely to have typical lupus symptoms, higher Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) scores (P = 0.002), and positive rate of anti-ribosomal P protein antibodies (P = 0.008). Patients with seizure were more likely to have higher SLEDAI-2K scores and positive anti-ß2GP1 than non-NPSLE patients. Sixteen patients died during follow-up. The most common cause of death was infection (37.5%). NPSLE significantly decreased survival rates of SLE patients. Patients with elevated serum creatinine (P = 0.001), hypocomplementemia (P = 0.031), and SLEDAI - 2K scores ≥ 15 (P = 0.014) had shorter survival periods. Eighty-two patients underwent detailed cranial MRI analysis; of these, 50 (61.0%) had abnormal results. Small vessel disease was the most common abnormal finding, followed by inflammatory-like lesions and large vessel disease. CONCLUSIONS: High disease activity and positive rate of anti-ribosomal P protein antibodies may be risk factors for NPSLE. NPSLE decreases survival rates of SLE patients. Renal insufficiency and high disease activity are predictive of poor prognoses for NPSLE patients.

Neuropsychiatric involvement in systemic lupus erythematosus: A review.

The neuropsychiatric involvement in systemic lupus erythematosus (NPSLE) is a challenge for clinicians, both at a diagnostic and therapeutic level. Although in 1999 the American College of Rheumatology (ACR) proposed a set of definitions for 19 NPSLE syndromes, with the intention of homogenizing the terminology for research purposes and clinical practice, the prevalence of NPSLE varies widely according to different series and is estimated to be between 37 and 95%. This is due to multiple factors such as the unalike definitions used, the diverse design of the studies, type of population, race, type and severity of symptoms, and follow-up of the different cohorts of patients with SLE. In recent years, some authors have tried excluding minor neuropsychiatric manifestations in order to try to reduce this wide variation in the prevalence of NPSLE since they are very prevalent in the general population; others authors have developed various models for the attribution of neuropsychiatric events to SLE that can assist clinicians in this diagnostic process, and finally, some authors developed and validated in 2014 a new algorithm based on the definitions of the ACR that includes the evaluation of the patient's lupus activity together with imaging techniques and the analysis of cerebrospinal fluid (CSF), with the aim of trying to differentiate the true neuropsychiatric manifestations attributable to SLE. In 2010, the European League Against Rheumatism (EULAR) developed recommendations for the management of NPSLE. We found abundant literature published later where, in addition to the recommendations for the management of the 19 NPSLE syndromes defined by the ACR, additional recommendations are given for other neurological and/or psychiatric syndromes, conditions, and complications that have been associated to SLE in recent years. We review below the diagnostic and therapeutic management of the different entities.

Neuropsychiatric Syndromes in Childhood-Onset Systemic Lupus Erythematosus: A Systematic Review and Meta-analysis.

OBJECTIVE: The aim of this study was to access the prevalence of 19 neuropsychiatric syndromes in childhood-onset systemic lupus erythematosus (cSLE), as defined by the American College of Rheumatology (ACR) in 1999, by performing a systematic review and meta-analysis of relevant publications. METHODS: A literature search from April 1999 to March 2018 identified studies investigating neuropsychiatric syndromes in cSLE patients, applying 1999 ACR Case Definitions, with a sample of at least 20 patients. Case reports, small case series, reviews, articles that did not use 1999 ACR case definitions, and those with adult SLE patients were excluded. The methodological quality of the studies was determined through the Loney quality assessment. Prevalence estimates with a 95% confidence interval were combined using random-effect (DerSimonian-Laird) models. RESULTS: A total of 143 articles were identified and 9 were included. In a population of 1463 cSLE patients, 351 (29.9%) presented 869 neuropsychiatric cSLE events (2.48 events/patient). The results for each syndrome were headache (52.2%), seizure disorders (48.6%), cognitive dysfunction (32.9%), mood disorder (28.3%), psychosis (22.7%), cerebrovascular disease (19.5%), acute confusional state (15.7%), movement disorder (9.4%), anxiety disorder (7.2%), aseptic meningitis (5.1%), mononeuropathy single/multiplex (4.9%), myelopathy (4.2%), demyelinating syndrome (3.2%), cranial neuropathy (2.7%), polyneuropathy (2.6%), Guillain-Barré syndrome (2.5%), autonomic disorder (1.9%), plexopathy (1.3%), and myasthenia gravis (1.3%). CONCLUSIONS: Neuropsychiatric syndromes are prevalent among cSLE patients. The most prevalent were headaches, seizures, cognitive dysfunction, mood disorders, and psychosis. Determining the prevalence of each neuropsychiatric syndrome in cSLE may improve clinical awareness of these potentially fatal and disabling conditions.

Unusual presentations in patients with systemic lupus erythematosus: a result of disease activity or something else?

Systemic lupus erythematosus can affect any organ or tissue, but skin manifestations, joint pain and fatigue are the most common symptoms. Two case reports in this issue describe patients with systemic lupus erythematosus who suffered severe internal organ manifestations.

Evolving phenotype of systemic lupus erythematosus in Caucasians: low incidence of lupus nephritis, high burden of neuropsychiatric disease and increased rates of late-onset lupus in the 'Attikon' cohort.

OBJECTIVE: This study aimed to analyse the phenotype of systemic lupus erythematosus (SLE) at first presentation and during follow-up in a newly established SLE cohort based at 'Attikon' University Hospital. The hospital combines primary, secondary and tertiary care for the region of Western Attica, Greece. METHODS: This study comprised a mixed prevalent and incident cohort of 555 Caucasian patients diagnosed with SLE according to American College of Rheumatology 1997 criteria and/or the Systemic Lupus Erythematosus International Collaborating Clinics (SLICC) 2012 criteria. Demographic and clinical characteristics, patterns of severity, treatments and SLICC damage index were recorded for each patient at the time of diagnosis and at last evaluation. RESULTS: The mean age at lupus diagnosis was 38.3 years (standard deviation = 15.6 years), with a median disease duration at last follow-up of two years (interquartile range 1-11). At initial presentation, the most common 'classification' manifestations were arthritis (73.3%), acute cutaneous lupus (65%) and unexplained fever (25%), while among symptoms not included in any criteria set, Raynaud's phenomenon (33%) was the most common. Kidney and neuropsychiatric involvement as presenting manifestations were present in 10.3% and 11.5% cases, respectively. Irreversible damage accrual was present in 17.8% within six months of disease diagnosis, attributed mainly to thrombotic and neuropsychiatric disease. At last evaluation, 202 (36.4%) patients had developed severe disease, of whom more than half were treated with pulse cyclophosphamide. CONCLUSION: In this cohort of Caucasian patients, lupus nephritis is not as common as in older cohorts, while neuropsychiatric disease is emerging as a major frontier in lupus prevention and care. These data may help to document changes in the natural history and treatment of SLE over time and may have implications for its early recognition and management.

Prevalence, outcome and prognostic factors of neuropsychiatric systemic lupus erythematosus: A real world single center study.

Objectives: To investigate the prevalence, outcome and prognostic factors of neuropsychiatric systemic lupus erythematosus (NPSLE).Methods: SLE inpatients from 2005 to 2016 were included. Information on survival duration and causes of death was collected. Data were analyzed using Kaplan-Meier curves, log-rank tests and Cox proportional hazards modeling.Results: Among 1589 SLE patients, 101 (6.4%) were diagnosed with NPSLE. The overall survival rates of the NPSLE patients were 89%, 85% and 84% at 1, 3 and 5 years, respectively. The standardized mortality ratio of NPSLE patients was 11.14. The most common cause of death was NPSLE related conditions (7, 47%), including intracranial hypertension syndrome, cerebrovascular disease and motor neuron disease. The following variables were associated with death: cardiac involvement, renal involvement, diffuse NPSLE, acute confusional state, more than one NPSLE manifestation, low lymphocyte count, elevated C-reactive protein, abnormal cerebrospinal fluid (CSF) and high systemic lupus erythematosus disease activity index. Acute confusional state (p = .001), elevated intracranial pressure (p = .010) and C-reactive protein (CRP) (p = .032) were independently predictive factors of death.Conclusion: Our study demonstrates an 11.14-fold increased mortality of NPSLE patients compared with general population. NPSLE related disorders are main causes of death. Acute confusional state is the most significant predictive factor for poor prognosis.

Characteristics and risk factors of an emergency department visit in patients with systemic lupus erythematosus.

This study aimed to examine the characteristics of patients with systemic lupus erythematosus (SLE) visiting the emergency department (ED) and the risk factors of an ED visit by these patients. This 4-year retrospective study was performed at a tertiary care center in Japan. We included all 205 patients with SLE who were treated in our outpatient clinic between April 1, 2008 and March 31, 2012 and divided them into two groups: those who visited the ED (the ED-user group) and those who did not (the ED-non-user group). We statistically compared the patient backgrounds and characteristics of the groups and identified the risk factors of an ED visit. Of all the patients, 118 visited the ED during study period and 87 did not. In total, 269 events were identified in the ED-user group. Of these, 91 (33.8%) were cases of infection, 32 (11.9%) were orthopedic problems, 32 (11.9%) were cases of gastrointestinal disease, 31 (11.5%) were cases of neurological disease, and 25 (9.3%) were cardiovascular events. Twenty-four events (8.9%) were due to SLE flares, of which ten (41.7%) were cases of neuropsychiatric lupus (NPSLE). The glucocorticoid dosage and the presence of a psychiatric illness, NPSLE, and lupus nephritis were higher among the ED-user group. Multivariate logistic regression analysis demonstrated high glucocorticoid dosage to be a risk factor of an ED visit. Among SLE patients, infections were the principal reason for visiting the ED. The most common reasons for an ED visit were common diseases rather than flares.

Chinese Systemic Lupus Erythematosus Treatment and Research Group (CSTAR) Registry XI: gender impact on long-term outcomes.

OBJECTIVE: The objective of this paper is to assess the role of gender on survival rate and causes of death and organ damage in systemic lupus erythematosus (SLE) patients in China from 2009 to 2015. METHODS: We conducted a multicenter cohort study to analyze the differences in outcome data between male and female SLE patients. A group of 1494 SLE patients who fulfilled the 1997 American College of Rheumatology SLE classification criteria were enrolled in the Chinese Systemic Lupus Erythematosus Treatment and Research Group (CSTAR) registry from April 2009 to February 2010. All enrolled patients were followed up at least once per year from 2009 to 2015. For patients who could not attend the outpatient clinic, follow-up was conducted by telephone interview. We collected demographic data, clinical manifestations and damage scores (System Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI)). Survival rates were evaluated using the Kaplan-Meier method. RESULTS: This study included 1352 women and 142 men. The five-year survival rates were 92.0% for men and 97.6% for women. The survival rates of males were significant lower than for females ( p = 0.019). Male patients received methylprednisolone pulse therapy and cyclophosphamide significantly more than female patients ( p = 0.010). During follow-up, 12 male patients and 66 female patients died. The most common cause of death was infection (41.7%) for men and active SLE disease (27.3%) for women. At the end of the study, the major accumulated organ damages included renal (8.5%) and musculoskeletal (7.7%), and nervous system (5.6%) for men and renal (8.8%) and musculoskeletal (6.7%) for women. There were no significant differences in SDI scores between the two groups at baseline and at the end of the study. CONCLUSIONS: Male SLE patients had lower survival rates than female patients. Male patients received more methylprednisolone pulse and cyclophosphamide therapy. The most common causes of death were infection for male patients and active SLE disease for female patients. The major accumulated organ damages were renal, musculoskeletal, and nervous system both for male and female SLE patients in China.

Epidemiology, characterization, and diagnosis of neuropsychiatric events in systemic lupus erythematosus.

INTRODUCTION: Neuropsychiatric systemic lupus erythematosus (NPSLE) is characterized by a heterogeneity of clinical manifestations. The absence of diagnostic criteria and the lack of clinical trials is a challenge in clinical practice. Areas covered: A literature review was performed to describe epidemiology, characterization (clinical, immunological, and imaging), diagnosis and treatment of NPSLE. Classification criteria have been the first step towards a uniform definition. More recently, different attribution models have been developed to help to determine if the NP event is due to SLE. Disease activity is a major risk factor for NP events. Cytokines and autoantibodies are associated with NP events, however, only a few studies have identified risk factors for individual NP events. Expert opinion: Further research needs to search for and validate biomarkers for NPSLE and individual NP events, including neuroimaging findings, attribution models, and serologic markers. This will be a fundamental step in planning randomized control trials in the treatment of NPSLE to improve outcome.

Long-Term Clinical Outcomes in a Cohort of Adults With Childhood-Onset Systemic Lupus Erythematosus.

OBJECTIVE: Childhood-onset systemic lupus erythematosus (SLE) is a severe, lifelong, multisystem autoimmune disease. Long-term outcome data are limited. This study was undertaken to identify clinical characteristics and health-related quality of life (HRQoL) of adults with childhood-onset SLE. METHODS: Patients participated in a single study visit comprising a structured history and physical examination. Disease activity (scored using the SLE Disease Activity Index 2000 [SLEDAI-2K]), damage (scored using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index [SDI]), and HRQoL (scored using the Short Form 36 Health Survey) were assessed. Medical records were reviewed. RESULTS: In total, 111 childhood-onset SLE patients were included; the median disease duration was 20 years, 91% of patients were female, and 72% were white. Disease activity was low (median SLEDAI-2K score 4), and 71% of patients received prednisone, hydroxychloroquine (HCQ), and/or other disease-modifying antirheumatic drugs. The vast majority of new childhood-onset SLE-related manifestations developed within 2 years of diagnosis. Damage such as myocardial infarctions began occurring after 5 years. Most patients (62%) experienced damage, predominantly in the musculoskeletal, neuropsychiatric, and renal systems. Cerebrovascular accidents, renal transplants, replacement arthroplasties, and myocardial infarctions typically occurred at a young age (median age 20 years, 24 years, 34 years, and 39 years, respectively). Multivariate logistic regression analysis showed that damage accrual was associated with disease duration (odds ratio [OR] 1.15, P < 0.001), antiphospholipid antibody positivity (OR 3.56, P = 0.026), and hypertension (OR 3.21, P = 0.043). Current HCQ monotherapy was associated with an SDI score of 0 (OR 0.16, P = 0.009). In this cohort, HRQoL was impaired compared to the overall Dutch population. The presence of damage reduced HRQoL scores in 1 domain. High disease activity (SLEDAI-2K score ≥8) and changes in physical appearance strongly reduced HRQoL scores (in 4 of 8 domains and 7 of 8 domains, respectively). CONCLUSION: The majority of adults with childhood-onset SLE in this large cohort developed significant damage at a young age and had impaired HRQoL without achieving drug-free remission, illustrating the substantial impact of childhood-onset SLE on future life.

Psychosis in Systemic Lupus Erythematosus: Results From an International Inception Cohort Study.

OBJECTIVE: To determine, in a large, multiethnic/multiracial, prospective inception cohort of patients with systemic lupus erythematosus (SLE), the frequency, attribution, clinical, and autoantibody associations with lupus psychosis and the short- and long-term outcomes as assessed by physicians and patients. METHODS: Patients were evaluated annually for 19 neuropsychiatric (NP) events including psychosis. Scores on the Systemic Lupus Erythematosus Disease Activity Index 2000, the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index, and the Short Form 36 (SF-36) were recorded. Time to event and linear regressions were used as appropriate. RESULTS: Of 1,826 SLE patients, 88.8% were female and 48.8% were Caucasian. The mean ± SD age was 35.1 ± 13.3 years, the mean ± SD disease duration was 5.6 ± 4.2 months, and the mean ± SD follow-up period was 7.4 ± 4.5 years. There were 31 psychotic events in 28 of 1,826 patients (1.53%), and most patients had a single event (26 of 28 [93%]). In the majority of patients (20 of 25 [80%]) and events (28 of 31 [90%]), psychosis was attributed to SLE, usually either in the year prior to or within 3 years of SLE diagnosis. Positive associations (hazard ratios [HRs] and 95% confidence intervals [95% CIs]) with lupus psychosis were previous SLE NP events (HR 3.59 [95% CI 1.16-11.14]), male sex (HR 3.0 [95% CI 1.20-7.50]), younger age at SLE diagnosis (per 10 years) (HR 1.45 [95% CI 1.01-2.07]), and African ancestry (HR 4.59 [95% CI 1.79-11.76]). By physician assessment, most psychotic events resolved by the second annual visit following onset, in parallel with an improvement in patient-reported SF-36 summary and subscale scores. CONCLUSION: Psychosis is an infrequent manifestation of NPSLE. Generally, it occurs early after SLE onset and has a significant negative impact on health status. As determined by patient and physician report, the short- and long-term outlooks are good for most patients, although careful follow-up is required.

Neuropsychiatric manifestations are not uncommon in Indian lupus patients and negatively affect quality of life.

Objectives The objectives of this paper are to look at the prevalence of neuropsychiatric manifestations and assess their impact on quality of life in North Indian lupus patients. Methods The study included consecutive patients with systemic lupus erythematosus (SLE) who were older than 18 years and met the SLICC 2012 criteria. A diagnosis of a neuropsychiatric syndrome was made as per ACR 1999 definitions. Manifestations occurring at any point in time after the diagnosis of SLE were considered if a reliable history and medical records were available. Quality of life was assessed by EuroQol-5D questionnaire. Means were compared by student t test for normally distributed data. Comparison of quality of life between groups was performed by the Kruskal-Wallis test and Mann-Whitney U test. Results This study included 101 patients of SLE with mean (±SD) age of 32.3 ± 10.0 years and a majority ( n = 92) were females. Mean (±SD) age of diagnosis of SLE was 27.8 ± 9.2 years and disease duration (after diagnosis) was 4.6 ± 4.5 years. Thirty-three patients had neuropsychiatric manifestations with a total of 42 events. The most common manifestation was headache (10%) followed by anxiety disorder (5%) and peripheral neuropathy (9%). Other NPSLE syndromes observed in the study are seizure (4%), cognitive dysfunction (4%), depression (4%), acute confusional state (2%), autonomic neuropathy (2%), movement disorder (1%), and mononeuritis multiplex (1%). On comparing the groups of NPSLE, nephritis, and neither, there was a significant difference in mobility, self-care, pain, and worry. On post hoc test, there was a significant difference between the NPSLE and neither group. Conclusion Neuropsychiatric manifestations significantly affect quality of life in North Indian SLE patients.