Late sequelae of systemic lupus erythematosus in children

Abstract

Objectives : Systemic lupus erythematosus classifield as one of the systemic inflammatory diseases of connective tissue, is a classic example of an autoimmune disease. Juvenile systemic lupus erythematosus contributes 5–10% of all cases of this disease. Based on 20-years follow up, the authors tried to assess the variety of clinical manifestations, course, prognosis and the most common sequelae of the disease in children. Material and methods : The study involved an analysis of the medical records of 138 patients. All the patients met 4 or more of the American College of Rheumatology Classification Criteria. The group of children was hospitalized in the Institute of Rheumatology in Warsaw from 1985 to 2005. The disease activity at its onset and during its further course was estimated according to the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Results : The assessment of the course of the disease in the examined period indicated that considerable improvement, evidenced by significant reduction or resolution of disease activity, was achieved in 85 children, while the disease progressed or remained unchanged despite the treatment in 47 children. Six cases resulted in the death of patients. The most frequent cause of death was sepsis. Conclusions : The course of SLE in children has changed over the last 20 years. The mortality rate has decreased considerably. Amelioration and remission are more frequent today. The average age of onset has increased by 2 years. The most frequent cause of death is general infections (previously it was renal failure). Currently the disease activity in the initial stage is lower, according to SLEDAI. In the initial stage of the disease there is a statistically significant decrease of frequency of occurrence of symptoms such as kidney involvement and epilepsy attacks. The increase in frequency of psychosis occurrence and the presence of anti-nDNA antibodies in blood serum is statistically significant.

Keywords

• juvenile systemic lupus erythematosus
• late consequences
• SLEDAI index
• SLICC/ACR Damage Index for SLE