Cognitive and Psychiatric Manifestations of Childhood-Onset Systemic Lupus Erythematosus

2010 ◽  
Author(s):  
Deborah M. Levy ◽  
Gail S. Ross
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Organ Damage ◽  
Multiple Organ ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Disease Manifestation ◽  
American College Of Rheumatology ◽  
Organ Systems ◽  
Similar Disease

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).

scholarly journals Oral lesions as a clinical sign of systemic lupus erythematosus

2018 ◽  
Vol 51 (3) ◽  
pp. 147
Author(s):  
Eliza Kristina M. Munthe ◽  
Irna Sufiawati
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Symptoms ◽  
Organ Damage ◽  
Multiple Organ ◽  
Suspected Case ◽  
Oral Lesions ◽  
Systemic Lupus ◽  
Organ Systems ◽  
High Degree

Background: Oral lesions represent one of the most important clinical symptoms of systemic lupus erythematosus (SLE), an autoimmune disease with a high degree of clinical variability rendering it difficult to arrive at a prompt and accurate diagnosis. There are many unknown causes and multiple organ systems involved, with the result that permanent organ damage may occur before treatment commences. Purpose: The purpose of this case report is to discuss the importance of recognizing the lesions related to SLE which may help dentists to make an early diagnosis. Case: A 17-year-old female patient was referred by the Internal Medicine Department with a suspected case of SLE. Prior to admittance to the hospital, the patient was diagnosed with tuberculosis. A subsequent extraoral examination revealed ulceration with a blackish crust on the upper lip. An intraoral examination showed similar ulceration covered with a blackish crust on the labial mucosa accompanied by central erythema in the hard palate. Blood tests indicated decreased levels of hemoglobin, hematocrit and platelets, but increased levels of leukocytes. A diagnosis of oral lesions associated with SLE and angioedema was formulated. Case management: The patient was given 1% hydrocortisone and vaseline album for extraoral lesions, while 0.2% chlorhexidine gluconate and 0.1% triamcinolone acetonide was used to treat intraoral lesions. An improvement in the oral lesions manifested itself after two weeks of treatment. Conclusion: Early detection of oral lesions plays a significant role in diagnosing SLE. It is important for the dentist to recognize the presentation of diseases that may be preceded by oral lesions. A multidisciplinary approach and appropriate referrals are necessary to ensure comprehensive medical and dental management of patients with SLE.

scholarly journals Cytokine Networks in Systemic Lupus Erythematosus

2010 ◽  
Vol 2010 ◽  
pp. 1-5 ◽  
Author(s):  
Hooi-Ming Lee ◽  
Hidehiko Sugino ◽  
Norihiro Nishimoto
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Cytokine Production ◽  
Organ Damage ◽  
Multiple Organ ◽  
Serum Cytokine ◽  
Systemic Lupus ◽  
Pathological Conditions ◽  
Cytokine Levels ◽  
Cytokine Networks

Systemic lupus erythematosus (SLE) is an autoimmune disease more prominent in women and characterized by multiple organ damage. Imbalance in cytokine production and cytokine levels correlates with SLE progression, making the understanding of SLE cytokine networks very important for SLE treatment strategy and drug development. In this article, we review cytokine networks that may be involved in the pathogenesis of SLE by briefly describing abnormal cytokine production and serum cytokine levels in SLE patients. We also focus on the pathological roles of cytokines and their interactions in immunoregulatory networks and suggest how their disturbances may implicate in pathological conditions in SLE. Finally, we further discuss the influence of estrogen on these cytokine networks.

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 ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Short Form ◽  
Organ Damage ◽  
Self Report ◽  
Systemic Lupus ◽  
Case Definitions ◽  
American College Of Rheumatology ◽  
Academic Center ◽  
Over Time

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.

scholarly journals The diagnostic accuracies of the 2012 SLICC criteria and the proposed EULAR/ACR criteria for systemic lupus erythematosus classification are comparable

Lupus ◽  
2019 ◽  
Vol 28 (6) ◽  
pp. 778-782 ◽  
Author(s):  
Ö Dahlström ◽  
C Sjöwall
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Diagnostic Sensitivity ◽  
Systemic Autoimmune Disease ◽  
Systemic Lupus ◽  
American College Of Rheumatology ◽  
Acr Criteria ◽  
Independent Evaluation ◽  
Organ Systems ◽  
Sensitivity Specificity

In a joint effort, the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) recently proposed new criteria for the classification of systemic lupus erythematosus (SLE) with the overarching goal to identify potential participants for clinical studies. Herein, we present the first independent evaluation of these criteria in comparison with older classification grounds using an adult Scandinavian study population of confirmed SLE cases and individuals with SLE-mimicking conditions. We included 56 confirmed SLE cases meeting the 1982 ACR criteria (ACR-82) and/or the Fries “diagnostic principle” (antinuclear antibodies on at least one occasion plus involvement of at least two defined organ systems) and 55 controls with possible systemic autoimmune disease, including the presence of any SLE-related autoantibody. The proposed EULAR/ACR criteria showed a diagnostic sensitivity of 93% (95% confidence interval (CI), 0.83–0.98) compared with 83% (95% CI, 0.72–0.91) for the updated ACR criteria from 1997. The diagnostic accuracy of all tested classification grounds was fairly similar, achieving approximately 85%. However, the disease specificity of the EULAR/ACR criteria reached only 73% (95% CI, 0.59–0.83), which was comparable with the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria, 75% (95% CI, 0.61–0.85), but clearly lower than for ACR-82, 94% (95% CI, 0.83–0.99). In this first independent evaluation of a limited number of cases, we found comparable results with respect to diagnostic sensitivity, specificity and accuracy regarding the SLICC-12 and the proposed EULAR/ACR classification criteria. However, their specificity for SLE appeared to be lower compared with ACR-82.

scholarly journals Clinical practice guidance for childhood-onset systemic lupus erythematosus—secondary publication

2021 ◽  
Author(s):  
Syuji Takei ◽  
Toru Igarashi ◽  
Tomohiro Kubota ◽  
Eriko Tanaka ◽  
Kenichi Yamaguchi ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Clinical Practice ◽  
Survival Rate ◽  
Lupus Erythematosus ◽  
Pediatric Rheumatology ◽  
Organ Damage ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Objective Evidence ◽  
Onset Systemic Lupus Erythematosus

ABSTRACT Childhood-onset systemic lupus erythematosus (cSLE) has been recognised as a more acute and severe autoimmune disease than adult-onset SLE. With the development of medications for the disease and supportive therapy, the mortality rate associated with cSLE has drastically improved; the 10-year survival rate among patients with cSLE between 1995 and 2006 in Japan was 98.3%. However, the 10-year survival rate without any permanent functional impairment remained low at 66.1%. Therefore, the current treatment goal for cSLE is to ensure that they can perform normal daily activities throughout their lives by preventing the occurrence and/or progression of organ damage. For this purpose, appropriate treatments and evaluations are required according to the severity and risk of organ damage; however, there are no established guidelines for cSLE. Therefore, the Pediatric Rheumatology Association of Japan and the Pediatric Rheumatology Subcommittee in the Japan College of Rheumatology developed a comprehensive guidance for clinical practice based on cSLE-related data collected from Japanese national surveys and relevant articles from both domestic and international sources. However, due to the lack of indications for defined and objective evidence quality levels, this guidance should be used on the basis of the judgement of the attending physicians for individual patients.

Childhood Systemic Lupus Erythematosus: Presentation, management and long-term outcomes in an Australian cohort

Lupus ◽  
2022 ◽  
pp. 096120332110697
Author(s):  
Megan P Cann ◽  
Anne M Sage ◽  
Elizabeth McKinnon ◽  
Senq-J Lee ◽  
Deborah Tunbridge ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Incidence Rate ◽  
Lupus Erythematosus ◽  
Renal Involvement ◽  
Damage Index ◽  
Organ Damage ◽  
Systemic Lupus ◽  
End Organ Damage ◽  
American College Of Rheumatology

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.

scholarly journals Inflammatory Cytokines in Systemic Lupus Erythematosus

2011 ◽  
Vol 2011 ◽  
pp. 1-14 ◽  
Author(s):  
Kim Ohl ◽  
Klaus Tenbrock
Keyword(s):  
Systemic Lupus Erythematosus ◽  
T Cell ◽  
Lupus Erythematosus ◽  
Therapeutic Potential ◽  
Unknown Origin ◽  
Organ Damage ◽  
Cell Phenotype ◽  
Systemic Lupus ◽  
Activation Induced Cell Death ◽  
Organ Systems

Systemic lupus erythematosus (SLE) is an autoimmune disease of unknown origin affecting virtually all organ systems. Beyond genetic and environmental factors, cytokine imbalances contribute to immune dysfunction, trigger inflammation, and induce organ damage. The key cytokine that is involved in SLE pathogenesis is interferon alpha. Interferon secretion is induced by immune complexes and leads to upregulation of several inflammatory proteins, which account for the so-called IFN signature that can be found in the majority of SLE PBMCs. Additionally IL-6 and IFN-y as well as T-cell-derived cytokines like IL-17, IL-21, and IL-2 are dysregulated in SLE. The latter induce a T-cell phenotype that is characterized by enhanced B-cell help and enhanced secretion of proinflammatory cytokines but reduced induction of suppressive T cells and activation-induced cell death. This paper will focus on these cytokines and highlights pathophysiological approaches and therapeutic potential.

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