Prospective Study of Neuropsychiatric Events in Systemic Lupus Erythematosus

2009 ◽  
Vol 36 (7) ◽  
pp. 1449-1459 ◽  
Author(s):  
JOHN G. HANLY ◽  
LI SU ◽  
VERN FAREWELL ◽  
GRACE McCURDY ◽  
LISA FOUGERE ◽  
...  

Objective.To prospectively examine neuropsychiatric (NP) events and their association with health related quality of life (HRQOL) over time in patients with systemic lupus erythematosus (SLE).Methods.In an observational cohort study from a single academic center, NP events and their attribution were identified at enrollment and at annual assessments for up to 7 years. NP events were characterized using the American College of Rheumatology case definitions; other variables were global SLE disease activity and cumulative organ damage. The outcomes of NP events were recorded and self-report HRQOL was measured with the mental (MCS) and physical (PCS) component summary scores of the Medical Outcomes Study Short Form-36.Results.There were 209 patients, 88% female and 92% Caucasian, with a mean (standard deviation) age of 43.7 (13.8) years. Followup was available in 175/209 (84%) patients. There were 299 NP events in 132/209 (63%) patients over a mean followup of 3.6 (2.5) years. Thirty-one percent of NP events in 54 patients were attributed to SLE. Multivariate analysis indicated lower MCS scores in patients with NP events compared to those without events (p < 0.001) regardless of attribution. The group means for PCS scores were significantly lower in patients with NP events (p < 0.001) regardless of attribution. There was no association between HRQOL and cumulative organ damage, nor between NP events and the progression of organ damage.Conclusion.The association of lower HRQOL with NP events over time, which is independent of progression in cumulative organ damage, emphasizes the persistent negative effect of NP events in the lives of patients with SLE.

Author(s):  
Fahima Hossain ◽  
Mohammad Delwer Hossain Hawlader ◽  
Dipak Kumar Mitra ◽  
Mohammad Hayatun Nabi ◽  
Md. Mujibur Rahman

Abstract Background Neuropsychiatric systemic lupus erythematosus (NPSLE) is well known for its varying presentations and poor outcomes, but little is evident about its distribution and characteristics among the Bangladeshi population. This study aimed to assess the pattern and prevalence of neuropsychiatric symptoms in female systemic lupus erythematosus (SLE) patients of Bangladesh. A retrospective study was conducted at a tertiary care hospital in Dhaka, Bangladesh, between January and December 2018. One hundred female SLE patients were included in the study purposively. Data were collected on sociodemographic and clinical characteristics of diagnosed SLE cases visiting the SLE clinic and indoor medicine department. Neuropsychiatric (NP) syndromes were defined according to the widely accepted American College of Rheumatology (ACR) nomenclature and case definitions. Results A total of 244 NP events were identified in fifty-five patients. Headache was the most frequent symptom (55%), followed by cognitive dysfunction (50%), anxiety (49%), psychosis (43%), seizure (23%), depression (17%), and cerebrovascular disease (ischemic type, 7%). The NP manifestations were more prevalent among urban residents (58.2%), younger patients (41.8%), and patients with graduate-level education (34.5%). Besides, young age at diagnosis (p = 0.038), Raynaud’s phenomenon (p = 0.015), other organ involvement (p < 0.001), and time of NPSLE development (p < 0.001) were found to be significantly associated with the development of these manifestations. Conclusion NP damage is prevalent among Bangladeshi female SLE patients (55%) with headache and cognitive dysfunction being the most common symptoms. Routine screening for neuropsychiatric symptoms among suspected SLE cases and further evaluation with a larger population are warranted.


Neurology ◽  
2001 ◽  
Vol 57 (3) ◽  
pp. 496-500 ◽  
Author(s):  
H. Ainiala ◽  
J. Loukkola ◽  
J. Peltola ◽  
M. Korpela ◽  
A. Hietaharju

Objective: To describe the prevalence of neuropsychiatric (NP) syndromes in a Finnish population of patients with systemic lupus erythematosus (SLE) and to classify them according to the recently developed American College of Rheumatology (ACR) nomenclature and case definitions for NPSLE.Methods: Cross-sectional, population-based study covering an area with 440,000 people. A total of 58 patients with a definite diagnosis of SLE and aged 16 to 65 years were found in the computerized database of the area hospitals. Of these, 46 (79%) agreed to participate. The diagnosis of various NP syndromes was based on clinical impression (H.A.) following history, examination, review of medical records, and neuropsychologic testing.Results: At least one NP syndrome was identified in 42 patients (91%). The most frequent manifestation was cognitive dysfunction (n = 37; 81%), followed by headache (n = 25; 54%) and mood disorder (n = 20; 43%). When mild NP syndromes (mild cognitive deficit, headache, mild depression, anxiety, electroneuromyography-negative polyneuropathy) were excluded, the prevalence of NPSLE dropped to 46%.Conclusions: According to the ACR nomenclature, there is a high prevalence of NP manifestations in a population-based sample of patients with SLE. Most NP syndromes were classified as minor; if they were excluded, the 46% prevalence of NPSLE would be slightly less than estimated in previous studies.


2011 ◽  
Vol 70 (6) ◽  
pp. 961-967 ◽  
Author(s):  
J G Hanly ◽  
M B Urowitz ◽  
D Jackson ◽  
S C Bae ◽  
C Gordon ◽  
...  

ObjectiveTo examine change in health-related quality of life in association with clinical outcomes of neuropsychiatric events in systemic lupus erythematosus (SLE).MethodsAn international study evaluated newly diagnosed SLE patients for neuropsychiatric events attributed to SLE and non-SLE causes. The outcome of events was determined by a physician-completed seven-point scale and compared with patient-completed Short Form 36 (SF-36) health survey questionnaires. Statistical analysis used linear mixed-effects regression models with patient-specific random effects.Results274 patients (92% female; 68% Caucasian), from a cohort of 1400, had one or more neuropsychiatric event in which the interval between assessments was 12.3±2 months. The overall difference in change between visits in mental component summary (MCS) scores of the SF-36 was significant (p<0.0001) following adjustments for gender, ethnicity, centre and previous score. A consistent improvement in neuropsychiatric status (N=295) was associated with an increase in the mean (SD) adjusted MCS score of 3.66 (0.89) in SF-36 scores. Between paired visits when the neuropsychiatric status consistently deteriorated (N=30), the adjusted MCS score decreased by 4.00 (1.96). For the physical component summary scores the corresponding changes were +1.73 (0.71) and −0.62 (1.58) (p<0.05), respectively. Changes in SF-36 subscales were in the same direction (p<0.05; with the exception of role physical). Sensitivity analyses confirmed these findings. Adjustment for age, education, medications, SLE disease activity, organ damage, disease duration, attribution and characteristics of neuropsychiatric events did not substantially alter the results.ConclusionChanges in SF-36 summary and subscale scores, in particular those related to mental health, are strongly associated with the clinical outcome of neuropsychiatric events in SLE patients.


Author(s):  
Deborah M. Levy ◽  
Gail S. Ross

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the production of autoantibodies directed against multiple organ systems. Individuals with SLE may have disease of only one organ system or of many organ systems at one time during a “flare” of disease activity. Systemic lupus erythematosus is characterized by multiple flares and remissions, and flares may lead to end-organ damage. The diagnosis of SLE is generally made by fulfilling four out of 11 of the American College of Rheumatology (ACR) 1997 Revised Classification Criteria for SLE (Hochberg 1997). Although the 1997 revised criteria have not been validated, the earlier 1982 criteria (Tan et al. 1982) have a sensitivity of 96% and specificity of 100%in childhood-onsetSLE(cSLE) (Ferraz et al. 1994). See Table 3.1 for these criteria. Systemic lupus erythematosus has a prevalence rate of 500 per million persons in the U.S. population (Klippel 1997) but may be as high as 130 per 100,000 persons (Uramoto et al. 1999). Approximately 15% of all SLE has its onset in childhood (prior to 18 years of age). Systemic lupus erythematosus occurs more commonly in non-Caucasians, with greater severity of SLE in children and adult Latino and African Americans than in non-Latino Caucasians (Michet et al. 1979).The female predominance of SLE in adulthood (10:1 female-to-male ratio) is less pronounced in childhood, and the ratio prior to puberty is more likely to be 5:1 or even 3:1.The majority of cSLE cases have onset in the peripubertal age period (10–15 years), which continues to suggest a link between estrogen and other hormones on the development of SLE. Childhood-onset SLE differs from adult-onset SLE in that the frequency of renal disease is higher, and the incidence and prevalence of neuropsychiatric SLE (NPSLE) is probably greater (Tucker et al. 1995; Sibbit et al. 2002). Children require aggressive immunosuppression, which on a dose per kilogram of body weight is generally higher than that required by an adult to treat a similar disease manifestation. Immunosuppression often includes corticosteroids. At least in adults, corticosteroids do not appear to cause cognitive impairment (Carbette et al. 1986; Denburg et al. 1997; Carlomagno et al. 2000).


2015 ◽  
Vol 2 (1) ◽  
pp. e000066-e000066 ◽  
Author(s):  
S. Al Sawah ◽  
X. Zhang ◽  
B. Zhu ◽  
L. S. Magder ◽  
S. A. Foster ◽  
...  

Lupus ◽  
2022 ◽  
pp. 096120332110697
Author(s):  
Megan P Cann ◽  
Anne M Sage ◽  
Elizabeth McKinnon ◽  
Senq-J Lee ◽  
Deborah Tunbridge ◽  
...  

Objectives Systemic Lupus Erythematosus (SLE) is a serious autoimmune disease often resulting in major end-organ damage and increased mortality. Currently, no data exists focussing on the presentation, long-term management and progression of SLE in the Australian paediatric population. We conducted the first Australian longitudinal review of childhood SLE, focussing on response to treatment and outcomes. Methods Detailed clinical and laboratory data of 42 children diagnosed with SLE before 16 years from 1998 to 2018 resident in Western Australia was collected. Data was collected at diagnosis and key clinical review time points and compared using the Systemic Lupus Collaborating Clinics (SLICC) and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) criteria. End organ damage was assessed against Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). Incidence rates of disease complications and end organ damage were determined. Results Of the 42 children, 88% were female with average age at diagnosis of 12.5 years. Indigenous Australians were over represented with an incidence rate 18-fold higher than non-Indigenous, although most children were Caucasian, reflecting the demographics of the Australian population. Median duration of follow-up was 4.25 years. On final review, 28.6% had developed cumulative organ damage as described by the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (incidence rate: 0.08/PY (95% CI 0.04–0.14)), and one child died. Twenty-nine children had renal involvement (incidence rate: 0.38/PY (95% CI 0.26–0.56)). Of the 27 patients with biopsy proven lupus nephritis, 70% had Class III or IV disease. Average length of prednisolone use from diagnosis was 32.5 months. Hydroxychloroquine ( n = 36) and mycophenolate mofetil ( n =21) were the most widely used steroid sparing agents. 61.9% received rituximab and/or cyclophosphamide. Conclusion This is the first longitudinal retrospective review of Australian children with SLE, with a markedly higher incidence in Indigenous children. Although improving, rates of end organ complications remain high, similar to international cohort outcomes. Longitudinal multi-centre research is crucial to elucidate risk factors for poor outcomes, and identifying those warranting early more aggressive therapy.


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