Clinical Analysis of 33 Cases of Pediatric Systemic Lupus Erythematosus
|School||Dalian Medical University|
|Keywords||children systemic lupus erythematosus clinical features therapy|
Objective:Pediatric systemic lupus erythematosus(pSLE),refers to the children (≦14)with systemic lupus erythematosus. Children make up 15% to 20% of all the SLE. In this article, we analyzed the initial manifestation, clinical manifestations, laboratory examinations, renal pathologic findings, treatment and prognosis through 33 cases of systemic lupus erythematous (SLE) in children, and studied the clinical features of pSLE.Methods:The clinical data of 33 cases of systemic lupus erythematosus in children admitted to our pediatric ward from June 2002 to March 2011 were reviewed retrospectively. Clincal data of the 33 children including the initial manifestation, clinical manifestations, laboratory examinations, renal pathologic findings, treatment and prognosis, involvement of organs and SLEDAI score were analysed detailly. We calculated 5-year survival of SLE children, and analyzed the treatment, effectiveness and prognosis of pSLE.Results:1.The male-to-female ratio wasl:7.5.The average age at presentation was 11.6±2.1 years. Skin eruption was the most common initial manifestation (66.67%) followed by fever(63%) and arthritic (50%).The commonly involved organs included kidney (87.88%) and hematologic system (81.82%).The most common renal pathology in lupus nephritis children is WHO classⅣ(42.86%). The patients usually have 3～6 organs involved (81.82%). Activity in disease (SLEDAI score) in the most cases were at moderate to severe levels (account for 84.85%). Among the laboratory parameters tested antinuclear antibody (ANA) was shown to have the highest positive rate of 90.91%, followed by anti-double stranded DNA (dsDNA) with a positive rate of 78.79%.2.Four of 33 patients were misdiagnosed at early stage of the disease.2 cases were characterized by repeated thrombocytopenic purpura while the other 2 cases were performance of nephritic syndrome.2 cases respectively on 4 years and 7 years later were diagnosed with SLE.3.Nineteen of 26 patients were lupus nephritis, treated combined with immunosuppressive agents for 3 months. The quantity of urinary protein, C3 and SLEDAI points were significantly improved (P<0.001, P<0.01, P<0.05 respectively). Compared with prednisone simple, the quantity of urinary protein reduced significantly (P<0.001). There were two patients died of unregular treatment. The 5-year survival of SLE children is 85.71%.4.Seventeen cases of activity in disease (SLEDAI score) were severe levels were treated with the methyl prednisone shock therapy. The clinical symptoms, signs and laboratory tests were improved obviously within 1-2 weeks.Conclusion:1.The manifestations of pediatric SLE are various and multisystem involvement is common. The commonly involved organs are kidney and hematologic system. Child patients with SLE are mainly severe cases.2.The initial manifestation of pediatric SLE are various, some patients only involved kidney or hematologic system early.lt is difficult to diagnosis at early stage.3.It is important for the patients with lupus nephritis to do renal biopsy early, and treat combined with various immunosuppressive agents regularly in order to improve prognosis and survival rate.4.Patients with SLE having multisystem involvement are critical cases and treated with the methyl prednisone shock therapy could control disease rapidly, protect involvement organs, reduce and prevent complications.