Lupus nephritis (including antiphospholipid antibody syndrome), pediatric

Abstract

Childhood-onset systemic lupus erythematosus (cSLE) has been defined as a subset of SLE with onset prior to 18 years of age. Around 15% of SLE patients present with nephritis before this age, and debatably these patients have a greater genetic component to their disease etiology, a more systemic involvement, and a more severe disease course. Patients develop their loss of tolerance to self at an earlier time than patients with adult-onset SLE, or at least their loss of tolerance progresses sooner to immune complex deposition and tissue injury. In this chapter, we review the joint approach of pediatric nephrologists and rheumatologists towards patients with cSLE and nephritis.We address the areas where evidence exists and the areaswherewemust rely on expert opinion or evidence based on primarily adult studies. Fortunately, 5- and 10-year mortality is lower than in adult-onset lupus nephritis, and patient and renal survival has improved over the years.However, relapses during the transition from adolescence to adulthood are common and cardiovascular and infectious complications are frequent in long-term survivors.