Approximately 20% of patients withy systemic lupus erythematosus (SLE) have disease which begins in childhood. Several series have reported that childhood-onset SLE (cSLE) is often more severe than adult disease with more pronounced clinical, immunologic and serologic abnormalities. In particular, renal and central nervous system (CNS) involvement tend to be more severe in pediatric than in adult patients.
The morbidity and mortality associated with SLE result from a combination of severe organ damage from disease, infection or side effects and complications of treatment. Since neither disease activity nor disease damage in SLE can be measured directly with any clinical sign or laboratory value, a number of disease indices have been developed that allow standardized comparison of SLE cohorts. Disease activity indices including SLEDAI, SLAM, BILAG and ECLAM have been validated in cSLE and none appears superior. The SLICC damage index was developed to measure permanent disease damage in adults with SLE and was subsequently validated in cSLE. It has been suggested that pediatric version should include additional domains addressing pediatric specific issues such as growth failure and delayed puberty, but both indices are reversible and they do not fulfil the definition of nonreversible organ damage.
Several predictors (risk factors) have been linked with poor prognosis in cSLE. Using the SLICC-DI scoring system for damage, it has been demonstrated that renal and/or CNS disease represent major determinants of morbidity and mortality in cSLE. Diffuse proliferative glomerulonephritis is most consistently linked with a poor prognosis if associated with hypertension. Other suggested risk factors associated with poor prognosis in cSLE include acute thrombocytopenia, young age at diagnosis, male sex and nonwhite (black, Asian and Hispanic) ethnicity. The presence of antiphospholipid antibodies may predispose to disease damage by increasing the risk for recurrent venous thrombosis, stroke and thrombocytopenia.
Similar to previous adult studies, it has been shown in cSLE that cumulative disease activity over time results in increased damage, and early introduction of therapy with immunosuppressive drugs might improve the outcome by preventing permanent damage. The exact impact of corticosteroid therapy on increased disease damage is still controversial, but patients may benefit from judicious use of corticosteroid with refined tapering schedules and early introduction of steroid-sparing drugs. It has been also demonstrated that frequent occurrence of severe, major disease flares requiring intravenous cyclophosphamide treatment represent a risk factor for damage, but not mild or moderate flares.
Disclosure of Interest None Declared
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