AB1030 NEUROPSYCHIATRIC MANIFESTATIONS IN CHILDHOOD-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS: RETROSPECTIVE STUDY IN ONE SINGLE CENTER

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.

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Clinical and laboratory manifestations of childhood and adult-onset systemic lupus erythematosus in Cipto Mangunkusumo Hospital

Paediatrica Indonesiana ◽

10.14238/pi43.6.2003.199-204 ◽

2016 ◽

Vol 43 (6) ◽

pp. 199

Author(s):

Syarif Faisal ◽

Arwin Akib ◽

Taralan Tambunan

Keyword(s):

Systemic Lupus Erythematosus ◽

Retrospective Study ◽

Gastrointestinal Tract ◽

Child Health ◽

Lupus Erythematosus ◽

Adult Onset ◽

Childhood Onset ◽

Systemic Lupus ◽

Pediatric Allergy ◽

Onset Systemic Lupus Erythematosus

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

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