Clinical features of systemic lupus erythematosus in children:a report of 47 cases

Objective To summarize the clinical and laboratory features of systemic lupus erythematosus(SLE) in children.Methods The clinical data of 47 children with systemic lupus erythematosus were reviewed retrospectively.Results There were three neonatal lupus syndrome(NLS) cases,with the symptoms of rashes,thrombocytopenia and atrioventricular block.Ten patients had mild NLS,27 had severe cases and seven had critical cases.The common clinical features included fever(57.4%),rash(53.2%) and arthritis(36.2%).The commonly involved organs included kidney(59.6%),hematologic system(46.8%) and lung(8.5%).The common multisystem involvements included kidney plus hematologic involvement(25.5%),kidney plus lung involvement(8.5%),and kidney plus nervous system involvement(4.3%).The laboratory findings included abnormal complete blood count(46.8%),hematuria(55.3%),proteinuria(59.6%),increased level of serum creatinine(4.3%),positive ANA(100%),positive anti-ds-DNA antibody(51.1%),positive anti-Smith antibody(31.9%),positive anti-SSA antibody(27.7%),and decreased serum levels of C3(91.4%).The renal pathology of 22 cases showed that 27.3% were mesangial lupus nephritis(WHO class Ⅱ),4.6% were focal segmental lupus golmerulonephritis(class Ⅲ),54.5% were diffuse proliferative lupus nephritis(class Ⅳ) and 13.6% were membranous lupus nephritis(class Ⅴ).Conclusion Child patients with SLE are mainly severe cases.Fever of unknown origin and(or) arthralgia,together with abnormal complete blood count,urine analysis,immunologic disorder,especially with positive ANA and decreased serum level of C3 can help to diagnose SLE in children early.Kidney is the most common involved organ in SLE patients.The most common renal pathology in lupus nephritis patients is WHO class Ⅳ.The focus of diagnosis and treatment in children with SLE should be on lupus nephritis.Patients with SLE having multisystem involvement are critical cases and should be treated promptly and properly.The examination of NLS is helpful in the diagnosis of the neonates with rashes,thrombocytopenia or atrioventricular block.