Pediatric Systemic Lupus Nephritis: Current Research in Treatment and Outcomes

Kathryn L. Ormsby

Systemic lupus erythematosus (SLE) is a serious and devastating autoimmune rheumatic disease affecting 50 to 100 per 100,000 people in the United States and Europe. SLE has increased threefold over the past 2 decades. Childhood onset of disease (before 19 years) represents 15 to 20% of cases with higher rates of disease reported in females between 12 to 16 years (Paut, Piram, Guillaume, & Tran, 2007). Childhood-onset disease is more aggressive than adult-onset with large studies demonstrating mortality rates almost 8 times greater for patients less than 24 years. The leading presenting symptoms of SLE are constitutional and non-specific such as fatigue, headache, weight loss or mood swings. These symptoms are also encountered in healthy adolescents, which is a primary reason for delay in diagnosis and treatment. Many children have long periods of illness before they receive appropriate treatment placing them at risk for advanced disease and poorer outcomes (Paut et.al.,2007). Lupus nephritis (LN) affects up to 80% of patients with SLE and is a main determinant of pediatric patient outcomes. End-stage renal disease develops in 10 to 15% of lupus nephritis patients with the remainder suffering from various stages of chronic renal impairment (Scheven & Bakkaloglu, 2009). Lupus nephritis is severe in childhood-onset SLE with aggressive renal disease and requirement for higher doses of steroid and immunosuppressive drug treatments. Longer life expectancy from improved chronic disease management has increased awareness of new long-term complications and chronic disease morbidity (Huang et aI., 2010; Pogmarutani, Alpert, & Miller, 2006). Providers are challenged in delivering care to these adolescents given their disease severity, cumulative treatment toxicity, and the psychosocial consequences of a chronic illness that occurs during a time ofadolescent physical, emotional, and social development (Paut et aI., 2007). Nurse practitioners need to maintain their role as primary care providers for children with SLE and LN to manage ongoing childhood medical care and chronic care of co-morbid conditions. Coordination of care by the NP in a multidisciplinary approach is critical to the management ofthe child with SLE. The NPs knowledge of the disease process, medications, and treatments utilized in acute management of SLE and LN are crucial to the on-going management of care along the illness trajectory. Nurse practitioners empower the child and family through education, counseling, and supportive care to promote maintenance ofoptimal function and quality of life.

Pediatric Systemic Lupus Nephritis: Current Research in Treatment and Outcomes

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