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Clinical and serological manifestations associated with interferon-α levels in childhood-onset systemic lupus erythematosus
Postal, Mariana;Sinicato, Nailu Angélica;Peli?ari, Karina Oliveira;Marini, Roberto;Lavras Costallat, Lilian Tereza;Appenzeller, Simone;
Clinics , 2012, DOI: 10.6061/clinics/2012(02)11
Abstract: objective: to determine the serum levels of interferon alpha in childhood-onset systemic lupus erythematosus patients, their first-degree relatives and healthy controls and to evaluate the associations between serum interferon alpha and disease activity, laboratory findings and treatment features. methods: we screened consecutive childhood-onset systemic lupus erythematosus patients in a longitudinal cohort at the pediatric rheumatology unit of the state university of campinas between 2009 and 2010. all patients demonstrated disease onset before the age of 16. disease status was assessed according to the systemic lupus erythematosus disease activity index (sledai) and systemic lupus international collaborating clinics/american college of rheumatology damage index (sdi). interferon alpha levels were measured using an enzyme-linked immunoabsorbent assay. results: we included 57 childhood-onset systemic lupus erythematosus patients (mean age 17.33±4.50), 64 firstdegree relatives (mean age 39.95±5.66), and 57 healthy (mean age 19.30±4.97) controls. serum interferon alpha levels were significantly increased in childhood-onset systemic lupus erythematosus patients compared to their firstdegree relatives and healthy controls. interferon alpha levels were significantly increased in patients with positive dsdna antibodies, patients with cutaneous vasculitis, patients with new malar rash and patients who were not receiving medication. interferon alpha levels correlated with c3 levels and systemic lupus erythematosus disease activity index scores. in addition, we observed an inverse correlation between patient age and interferon alpha levels. conclusion: interferon alpha may play a role in the pathogenesis of childhood-onset systemic lupus erythematosus, especially in cutaneous manifestations and dsdna antibody formation. the observation that interferon alpha levels are increased in patients who are not taking medication should be investigated in longitudinal studies to determ
Damage index in childhood-onset systemic lupus erythematosus in Egypt
Samia Salah, Hala M Lotfy, Abir N Mokbel, Ahmed M Kaddah, Nouran Fahmy
Pediatric Rheumatology , 2011, DOI: 10.1186/1546-0096-9-36
Abstract: A total of 148 patients with jSLE have been followed in the pediatric rheumatology clinic and section at Cairo University. These patients were evaluated by retrospective chart review. The organ system damage due to SLE was measured using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). Risk factors for damage were also studied including demographic criteria as well as clinical and laboratory manifestations.Overall, 43.9% of the patients had damage within a mean of 6.57 ± 3.59 years of disease diagnosis. Neuropsychiatric (NPS-21%) and renal (16.9%) system involvement were observed most frequently, followed by cardiovascular (11.5%), skin (9.5%), pulmonary (6.1%), and ocular (4.8%), with a mean SDI score of 0.93 ± 1.37. In our study, the presence of neuropsychiatric manifestations at diagnosis showed the strongest association with the presence of later disease damage.The number of SLE diagnostic criteria at presentation was strongly associated with the total SDI score, and the renal damage was significantly more prevalent in patients with age at disease diagnosis below 10 years of age. A higher mean disease duration was found in patients with musculoskeletal damage.We found that cumulative organ damage, as measured by the SDI, was present in 43.9% of Egyptian patients with juvenile-onset SLE. The damage was significantly more likely in patients who had more SLE diagnostic criteria at time of disease presentation and NPS manifestations at the time of diagnosis.Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by autoantibodies directed against nuclear antigens and causing a variety of clinical and laboratory abnormalities [1]. SLE may involve multiple organs, causing significant morbidity and mortality in adults, adolescents, and children [2].Over the last few decades, there has been a remarkable improvement of survival among patients with juvenile-onset systemic lupus erythematos
Analysis of Gender Differences in Genetic Risk: Association of TNFAIP3 Polymorphism with Male Childhood-Onset Systemic Lupus Erythematosus in the Japanese Population  [PDF]
Keisuke Kadota, Masaaki Mori, Masakatsu Yanagimachi, Takako Miyamae, Takuma Hara, Taichi Kanetaka, Tomo Nozawa, Masako Kikuchi, Ryoki Hara, Tomoyuki Imagawa, Tetsuji Kaneko, Shumpei Yokota
PLOS ONE , 2013, DOI: 10.1371/journal.pone.0072551
Abstract: Background Systemic lupus erythematosus (SLE) is a systemic multisystem autoimmune disorder influenced by genetic background and environmental factors. Our aim here was to replicate findings of associations between 7 of the implicated single nucleotide polymorphisms (SNPs) in IRF5, BLK, STAT4, TNFAIP3, SPP1, TNIP1 and ETS1 genes with susceptibility to childhood-onset SLE in the Japanese population. In particular, we focused on gender differences in allelic frequencies. Methodology/Principal Findings The 7 SNPs were genotyped using TaqMan assays in 75 patients with childhood-onset SLE and in 190 healthy controls. The relationship between the cumulative number of risk alleles and SLE manifestations was explored in childhood-onset SLE. Logistic regression was used to test the effect of each polymorphism on susceptibility to SLE, and Wilcoxon rank sum testing was used for comparison of total risk alleles. Data on rs7574865 in the STAT4 gene and rs9138 in SPP1 were replicated for associations with SLE when comparing cases and controls (corrected P values ranging from 0.0043 to 0.027). The rs2230926 allele of TNFAIP3 was associated with susceptibility to SLE in males, but after Bonferroni correction there were no significant associations with any of the other four SNPs in IRF5, BLK, TNIP1 and ETS1 genes. The cumulative number of risk alleles was significantly increased in childhood-onset SLE relative to healthy controls (P = 0.0000041). Male SLE patients had a slightly but significantly higher frequency of the TNFAIP3 (rs2230926G) risk allele than female patients (odds ratio [OR] = 4.05, 95% confidence interval [95%CI] = 1.46–11.2 P<0.05). Conclusions Associations of polymorphisms in STAT4 and SPP1 with childhood-onset SLE were confirmed in a Japanese population. Although these are preliminary results for a limited number of cases, TNFAIP3 rs2230926G may be an important predictor of disease onset in males. We also replicated findings that the cumulative number of risk alleles was significantly increased in childhood-onset SLE.
Late onset systemic lupus erythematosus
Ivone Minhoto Mein?o,Emília Inoue Sato
Einstein (S?o Paulo) , 2008,
Abstract: Systemic lupus erythematosus starting beyond the age of 50 yearsis considerate as a late onset disease, and constitutes a subgroupwith some demographic, clinical and laboratory characteristics, inagreement with former studies. We performed a review searching onPubMed using key words present in the title. In this review articlespublished in the last ten years were included besides older ones includedin the rheumatology textbooks. Since the decade of the 1960sthere was the concept that late onset systemic lupus erythematosushad a benign course with less gender difference comparing to theyoung adult onset disease. More recent studies continue confi rmingthe lower predominance in female than male as compared to theyounger onset disease. However, several more recent studies evaluatingethnically different populations showed that the late onset diseasehad worst prognosis than published previously, due to comorbiditiesfrequent in this age group that can infl uence their healthy status. Themost frequent causes of mortality are infection followed by pulmonaryand coronary diseases. The lower frequency of hypocomplementemiain late onset disease continues to be found, however, renal involvementis now considered similar to that found in young adult disease.There is consensus that late onset disease affects less frequently theskin, especially malar rash and photosensitivity, however it seemsthat the course is not as benign as reported in previous studies.
Therapeutic effects of DNA immunoadsorption therapy on severe systemic lupus erythematosus

- , 2015,
Abstract: 摘要目的:探讨DNA免疫吸附治疗重症系统性红斑狼疮(SLE)的临床有效性、安全性。方法:收集重症SLE患者20例,分为观察组(8例)和对照组(12例)。观察组采用连续性血液净化联合DNA免疫吸附配合泼尼松片、环磷酰胺治疗,对照组采用甲强龙冲击后,给予泼尼松片联合环磷酰胺治疗。比较2组患者治疗前及治疗12周、24周SLEDAI评分及ANA、抗ds??DNA抗体滴度,IgG,IgA,IgM,C3,C4,肾功能等指标。结果:与对照组比较,治疗12周观察组SLEDAI评分、ANA与抗ds??DNA抗体滴度下降更快(P均<0.05),24周时观察组ANA滴度仍能维持在较低水平(P均<0.05)。治疗24周,2组患者肾功能均稳定。观察组除1例发生重症感染,无精神异常、心律失常等不良反应发生。结论:DNA免疫吸附治疗重症SLE安全、有效。
AbstractAim: To investigate the clinical efficacy and safety of DNA immunoadsorption on patients with severe systemic lupus erythematosus(SLE).Methods: Twenty cases of severe SLE were allocated into 2 groups; observation group(n=8) received continuous blood purification, DNA immunoadsorption and glucocorticoid treatment,and control group (n=12) received cyclophosphamide combined with glucocorticoid treatment. Before and at 12 and 24 weeks after the therapy, SLEDAI score, autoantibodies(ANA) and anti??dsDNA autoantibody titer, IgG, IgA, IgM levels, C3 and C4 and renal function parameters were assessed. Results: At 12 weeks after the treatment, the SLEDAI score, ANA and anti??dsDNA autoantibody titer in the observation group decreased more significantly than control group; at 24 weeks after the treatment, the ANA titer in the observation group still maintained in a low level(P<0.05). The renal function were stable in two groups during 24 weeks. There were no obvious side effects except for a case of severe infection. Conclusion: DNA immunoadsorption in treatment of severe SLE is effective and safe
Antiphospholipid syndrome in childhood onset systemic lupus erythematosus  [cached]
Danda D,Mathew A,Thomas K
Indian Journal of Medical Sciences , 2004,
Extent of Subclinical Pulmonary Involvement in Childhood Onset SystemicLupus Erythematosus in the Sultanate of Oman
Eiman Abdulla,Reem Abdwani,Ibrahim Al-Zakwani,Sawsan Baddar
Oman Medical Journal , 2012,
Abstract: Objectives: The aim of this study was to investigate the frequencyof pulmonary function abnormalities in clinically asymptomaticchildren with Systemic Lupus Erythematosus and to determinethe relationship of these abnormalities to clinical, laboratory, andimmunological parameters as well as to disease activity.Methods: Forty-two children with childhood onset SystemicLupus Erythematosus were included in this study. Demographic,clinical, laboratory and immunological parameters, as well asdisease activity were assessed. Pulmonary function tests (PFT)were performed routinely to screen for subclinical lung disease.Results: Out of the 42 children, 19 0(n=8) had clinical evidence ofpulmonary involvement. The patients with no clinical evidence ofpulmonary involvement (n=34) represent the study cohort. Fromour cohort of patients with no clinical evidence of pulmonaryinvolvement 79 0(n=27) had PFT abnormality; including 62 =21) had reduced FVC, 71 0(n=24) had reduced FEV1, and67 0(n=12) had reduced DLCO. Similarly, 56 0(n=15) hada restrictive PFT pattern, and 2.6 0(n=2) had an obstructivePFT pattern, while 33 0(n=7) had an isolated impairment ofdiffusion capacity. Due to small sample size; it was not possibleto find a statistically significant difference between the cohort ofasymptomatic SLE patients with abnormal PFT findings (n=27)and those with normal PFT findings (n=7) in terms of clinical,laboratory, immunological or disease activity index score.Conclusion: Subclinical lung disease, as demonstrated byabnormal PFT in patients with normal radiographs, may becommon but should be interpreted with caution as an early signof lung disease. Although PFT studies do not correlate well withpulmonary symptoms in patients with childhood onset SLE,they nevertheless provide objective quantification of the type andseverity of the functional lesions.
Unusual presentation of childhood Systemic Lupus Erythematosus
Sathish Kumar, Indira Agarwal
Pediatric Rheumatology , 2007, DOI: 10.1186/1546-0096-5-20
Abstract: Bullous systemic lupus erythematosus (SLE) is a rare, distinctive subepidermal blistering disorder that occurs in systemic lupus erythematosus [1]. It is characterized clinically by a pemphigoid-like eruption with tense fluid-filled vesicles and bullae, often with a background of maculopapular or urticated erythema. It can affect any area of the body, including non-sun-exposed sites and the mucous membranes. Pruritus is usually present in variable severity. The lesions form erosions and crusts before healing, usually but not invariably without scarring. Treatment with dapsone results in promising results. We describe a 13 years old girl who presented with bullous SLE.A 13-year-old girl presented with recurrent fever associated with increasing fatigue, arthraliga, hair loss and erythematosus bullous lesions over face, neck and extremities of 2 months duration. She also had one episode of generalised tonic clonic seizures prior to admission.She was treated with oral antibiotics and topical hydrocortisone for her skin lesions. On examination she had numerous bullae and vesicles on a background of urticated plaques affecting the back, abdomen, neck and flexures of the arm and groin regions (Fig. 1). Nikolsky's sign was negative. Erosions with crusts characterized older lesions. No scarring was seen at the sites of healed lesions. There were no mucosal erosions or blisters. No malar rash or oral ulcers. No raynaud's phenomenon. Cardiovascular and respiratory system examination were within normal limits. Abdominal examination did not reveal hepatosplenomegaly. Her fundoscopy did not reveal evidence of hypertensive encephalopathy or vasculitis. Otherwise neurologically she was normal.Investigations revealed hemoglobin was 90 g/L, white blood cell was 21 × 109/L with neutrophils of 2.5 × 109/L, lymphocytes of 1 × 109/L, bands 0.03 × 109/L. Her platelets were 20 × 109/L. Her INR and PTT were within normal limits. ESR was 55 mm at 1 hour and CRP was negative. Her serum creati
Lupus erythematosus profundus  [cached]
Aggarwal Kamal,Jain V,Dayal Surbhi
Indian Journal of Dermatology, Venereology and Leprology , 2002,
Abstract: A case of lupus erythematosus profundus, with associated mastitis, but without any lesions of discoid lupus erythematosus or systemic lupus erythematosus is being reported.
Demographics and presenting clinical features of childhood systemic lupus erythematosus
G Faller, UK Kala, PD Thomson, D Hahn
Journal of Endocrinology, Metabolism and Diabetes of South Africa , 2005,
Abstract: Objectives: To review the presentation and characteristics of children with systemic lupus erythematosus (SLE). Methods: The records of children with sufficient American College of Rheumatology (ACR) criteria for SLE treated by the renal units of the Johannesburg and Chris Hani Baragwanath hospitals, and the arthritis clinic of the Johannesburg Hospital between January 1974 and March 2000 were reviewed. The clinical presentation, age distribution and race were examined. Results: A total of 36 children met the criteria. There were 26 girls and 10 boys, with a mean age of 11.5 and 10.2 years respectively. The male-to-female ratio was 1:2.6 overall, with a ratio of 1:1.2 under 10 years and 1:4 over 10 years. There were 15 white, 2 Indian and 5 coloured patients. The 14 black patients all presented after 1986. Rashes were found to be the commonest clinical feature present at the time of diagnosis, followed by polyarthritis and renal pathology. Constitutional symptoms were common, as were generalised lymphadenopathy and hepatosplenomegaly, while neurological, pulmonary and cardiac signs and symptoms were less common. Renal disease was present in 58% of patients on presentation. Conclusion: There is a diverse array of presenting features in childhood SLE. There has been increased recognition of the disease in young black South Africans since 1986. Journal of Endocrinology, Metabolism and Diabetes of South Africa Vol. 10(2) 2005: 64-68
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