[Clinical features, treatment and follow-ups of childhood systemic lupus erythematosus]

Abstract

Objective: To summarize the characteristics of clinical features, laboratory tests, treatments and outcomes of childhood systemic lupus erythematosus (SLE).

Methods: A retrospective study was conducted for 130 cases of first-confirmed childhood SLE hospitalized from 2001 to 2008. Their clinical data of initial manifestations, system involvements, laboratory tests, treatments and outcomes were recorded and analyzed. Among them, there were 25 males and 105 females with a gender ratio of 1: 4.2. The youngest was 4 years old and the oldest 16 years old. And the mean age of onset was 11.5 ± 2.2 years.

Results: The initial manifestations included fever (66%), rash (62.33%) and fatigue (47.33%). And the cutaneous-mucosal system (80%), hematological system (70.77%) and kidney (66.15%) were frequently involved. Anemia (64.62%) and urine protein positivity (66.15%) were the most common abnormalities. All patients had positive ANA. The positive rate of anti-dsDNA was 88.37%. Six patients had antiphospholipid syndrome (APS). The levels of IgM-aCL and IgM-anti-β(2)-GP-Iwere moderately or higher positive. Glucocorticoid was the first-line medication. All patients took steroids. Different immunodepressants were also used depending on the severity of illness. The effective rates of one month and one year were 80.7% and 95.1% respectively. Six patients died.

Conclusion: The patients with serious organ involvements as the initial manifestations end to have a worse prognosis. Cutaneous-mucosal system, hematological system and kidney are the most frequently involved. Most patients have a high level of ANA titer. IgM-aCL and IgM-anti-β(2)-GP-Iare sensitive for diagnosing antiphospholipid syndrome. Early diagnosis, appropriate treatment and regular follow-ups may help to improve the clinical prognosis of SLE.