Clinical and immunological aspects and outcome of a Brazilian cohort of 414 patients with systemic lupus erythematosus (SLE): comparison between childhood-onset, adult-onset, and late-onset SLE

Lupus ◽  
2015 ◽  
Vol 25 (4) ◽  
pp. 355-363 ◽  
Author(s):  
M M das Chagas Medeiros ◽  
M Campos Bezerra ◽  
F N Holanda Ferreira Braga ◽  
M R Melo da Justa Feijão ◽  
A C Rodrigues Gois ◽  
...  
2016 ◽  
Vol 43 (8) ◽  
pp. 1490-1497 ◽  
Author(s):  
Young Bin Joo ◽  
So-Yeon Park ◽  
Soyoung Won ◽  
Sang-Cheol Bae

Objective.To compare clinical features and mortality between childhood-onset systemic lupus erythematosus (cSLE) and adult-onset SLE (aSLE) in a prospective single-center cohort.Methods.A total of 1112 patients with SLE (133 cSLE and 979 aSLE) were enrolled and followed from 1998 to 2012. The 2 groups were compared regarding American College of Rheumatology (ACR) classification criteria for SLE, autoantibodies, disease activity measured by the Adjusted Mean SLE Disease Activity Index (AMS), damage measured by the Systemic Lupus International Collaborating Clinics/ACR Damage Index (SDI), and medication. The standardized mortality ratio (SMR) was calculated. Predictors of mortality in SLE were evaluated using Cox proportional hazard models.Results.After a mean followup of 7.6 years, patients with cSLE had a higher number of cumulative ACR criteria and a higher AMS (p < 0.001 each), but there was no difference in SDI (p = 0.797). Immunosuppressants were used more frequently by patients with cSLE (p < 0.001). The SMR of cSLE was 18.8 (95% CI 8.6–35.6), significantly higher than that of aSLE (2.9, 95% CI 2.1–3.9). We found cSLE to be an independent predictor of mortality (HR 3.6, p = 0.008). Moreover, presence of hemolytic anemia (7.2, p = 0.034) and antiphospholipid antibody (aPL; 3.8, p = 0.041) increased the magnitude of risk of early mortality more in the patients with cSLE than in those with aSLE.Conclusion.The clinical course of cSLE as measured by number of clinical manifestations and disease activity is worse than that of aSLE. Also, cSLE patients with hemolytic anemia and aPL are at greater risk of death than patients with aSLE who have those features.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 612.2-613
Author(s):  
O. Iaremenko ◽  
D. Koliadenko ◽  
I. Matiyashchuk

Background:Systemic lupus erythematosus (SLE) predominantly develops in women of child-bearing age. However, nearly 20% of cases present during childhood, generally after puberty (juvenile-onset SLE, JSLE). On the other hand, 10-20% of patients develop SLE after the age of 45-50 years (late-onset SLE, LSLE) [1]. It is known that age at disease onset can influence the clinical presentation and course of SLE, but the findings are not always consistent across the studies [2].Objectives:The aim of this study was to evaluate the spectrum of clinical manifestations and autoantibody profile in patients with SLE in the central region of Ukraine regarding age at onset.Methods:The study included 258 SLE patients before starting an adequate therapy, comprising 225 females (87.2%) and 33 males (12.8%). The median age at SLE onset was 28 (20-39) years. The patients were classified into 3 groups: I – age at SLE onset ≤18 years (JSLE; n=52; 20.2%), II – SLE onset at age 19-44 years (adult-onset SLE, ASLE; n=161; 62.4%), III – age at disease onset ≥45 years (LSLE; n=45; 17.4%). The clinical and demographic data, SLE Disease Activity Index (SLEDAI), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and autoantibody profile were analyzed. Quantitative and categorical data were compared using Kruskal-Wallis test and chi-square test, respectively.Results:There was a difference in prevalence of malar rash between the groups (p=0.022): it was more common in JSLE (40.4%) and ASLE (34.4%) than in LSLE patients (15.6%; p=0.04 and 0.05, respectively). Similar distribution was found for renal involvement: JSLE and ASLE patients presented higher rates of nephritis (55.8% and 49.4%, respectively) than LSLE patients (23.8%; p=0.012 and 0.014, respectively). But the groups did not differ significantly with regard to nephrotic syndrome (p=0.224). ASLE was associated with more frequent alopecia (38.8%) comparing with JSLE (19.2%; p=0.04). Moreover, ASLE patients also had the highest frequency of lymphadenopathy (56.3%) whereas in LSLE it was observed only in 25.0% of patients (p=0.001). Serositis was more common in LSLE (54.5%) and ASLE (43.8%) than in JSLE (23.1%; p=0.011 and 0.034, respectively). Although secondary Sjögren’s syndrome was more frequently observed in ASLE (7.6%) and LSLE (7.3%) than in JSLE (0.0%), the difference did not achieve statistical significance (p=0.157). Also, no differences were observed in the occurence of arthritis, pulmonary and neurological manifestations, constitutional symptoms, SLEDAI score among the groups. Median CRP level in LSLE was significantly higher (14.0 (1.1-46.4) mg/L) than in JSLE (0.7 (0.0-12.0) mg/L) (p<0.05). But all groups did not differ significantly with regard to ESR levels. When differences in antinuclear antibodies were analyzed, we disclosed that the frequency of anti-dsDNA positive results was significantly higher in JSLE (68.6%) and ASLE (70.1%) patients when compared with that found in LSLE patients (31.3%) (p=0.016 and 0.001, respectively). There were no significant differences between groups with regard to positivity for other antibodies (anti-Sm, -Ro, -La, -RNP, antiphospholipid antibodies).Conclusion:JSLE and ASLE patients are more likely to have malar rash, nephritis and anti-dsDNA positivity. Alopecia and lymphadenopathy are most frequent in ASLE patients. JSLE are far less likely to have serositis than any other group. Patients with LSLE demonstrate comparatively low frequency of major organ involvement, but they have higher levels of CRP.References:[1]Ambrose N., et al. Differences in disease phenotype and severity in SLE across age groups. Lupus. 2016;25(14):1542-1550.[2]Livingston B., et al. Differences in autoantibody profiles and disease activity and damage scores between childhood- and adult-onset systemic lupus erythematosus: a meta-analysis. Seminars in Arthritis and Rheumatism. 2012;42(3):271-280.Disclosure of Interests:None declared


2016 ◽  
Vol 43 (6) ◽  
pp. 199
Author(s):  
Syarif Faisal ◽  
Arwin Akib ◽  
Taralan Tambunan

Background Childhood-onset SLE is more severe than the adult type.Objective To compare the clinical and laboratory manifestationsof childhood- and adult-onset systemic lupus erythematosus (SLE).Methods A retrospective study on child and adult SLE patientswas conducted in the Division of Pediatric Allergy and Immunol-ogy, Department of Child Health and Department of Internal Medi-cine, Medical School, University of Indonesia/CiptoMangunkusumoHospital (FKUI/RSCM) Jakarta.Subjects One hundred and twenty-nine subjects met the studycriteria consisting on 54 childhood- onset (41.9%), 75 adult-onset(58.1%), and 122 (94.6%) females and 7 (5.4%) males from Janu-ary 1995 until December 2000.Results Fever, arthralgia and vasculitis were the most frequentsigns found in both childhood-onset and adult-onset SLE. The liver,spleen, lymph nodes, cardiovascular, gastrointestinal tract, andeyes were the organs involved and significantly had a larger pro-portion in the childhood-onset. Decrease of C3 was more frequentlyfound in the childhood-onset. Out of 17 childhood-onset patientswho died, 13 died at the age of 0-11 year-old. Two of the adult-onset patients also died.Conclusion Childhood-onset SLE had some different character-istics compared to adult form


Lupus ◽  
2021 ◽  
pp. 096120332199192
Author(s):  
Kumutnart Chanprapaph ◽  
Ittipon Tubtieng ◽  
Nathathai Pratumchat ◽  
Kunlawat Thadanipon ◽  
Ploysyne Rattanakaemakorn ◽  
...  

Background Age at disease onset may modulate systemic lupus erythematosus (SLE), but its relation to cutaneous/extracutaneous manifestation remains understudied. Objective To compare the cutaneous, systemic features, laboratory characteristics, and disease severity between late- and adult-onset SLE patients Methods Analyses of the cutaneous, systemic involvement, laboratory investigations, SLE disease activity index 2000 (SLEDAI-2K), and disease damage were performed to compare between groups. Results Of 1006 SLE patients, 740 and 226 had adult- (15–50 years) and late-onset (>50 years), respectively. Among 782 with cutaneous lupus erythematosus (CLE), acute CLE (ACLE) and chronic CLE (CCLE) were more common in the adult- and late-onset SLE, respectively ( p = 0.001). Multivariable logistic regression analysis demonstrated that male patients and skin signs, including papulosquamous subacute CLE, discoid lupus erythematosus, and lupus profundus, were associated with late-onset SLE (all p < 0.05). Late-onset SLE had lower lupus-associated autoantibodies, and systemic involvement (all p < 0.05). ACLE, CCLE, mucosal lupus, alopecia, and non-specific lupus were related to higher disease activity in adult-onset SLE (all p < 0.001). There was no difference in the damage index between the two groups. Conclusions Late-onset SLE had a distinct disease expression with male predominance, milder disease activity, and lower systemic involvement. Cutaneous manifestations may hold prognostic values for SLE.


2008 ◽  
Vol 58 (2) ◽  
pp. 556-562 ◽  
Author(s):  
Hermine I. Brunner ◽  
Dafna D. Gladman ◽  
Dominique Ibañez ◽  
Murray D. Urowitz ◽  
Earl D. Silverman

2016 ◽  
Vol 36 (10) ◽  
pp. 1431-1437 ◽  
Author(s):  
Miguel Ángel Saavedra ◽  
Dafhne Miranda-Hernández ◽  
Antonio Sánchez ◽  
Sara Morales ◽  
Pilar Cruz-Domínguez ◽  
...  

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