Sistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSPWENDERFER, S.MASON, S.BERNAL, C.SILVA, C. A. A. da2023-12-042023-12-042022Wenderfer, S.; Mason, S.; Bernal, C.; da Silva, C. A. A.. Lupus Nephritis. In: . Pediatric Nephrology: Eighth Edition: SPRINGER INTERNATIONAL PUBLISHING, 2022. p.507-539.978-303052719-8; 978-303052718-1https://observatorio.fm.usp.br/handle/OPI/57211Childhood-onset systemic lupus erythematosus (cSLE) is a subset of SLE with onset prior to 16-18 years of age. Around 15% of patients with SLE present in childhood; patients with cSLE have a greater genetic component to their disease, more systemic involvement, and more severe disease course including higher risk for developing nephritis. Patients with cSLE develop loss of tolerance to self at an earlier time than patients with adult-onset SLE, or loss of tolerance progresses sooner to autoantibody production, immune complex deposition, and immune-mediated tissue injury. We review the diagnosis, classification criteria, and management of kidney disease in cSLE, by pediatric nephrologists and rheumatologists. Although the evidence basis for management often primarily relies on adult studies, there is growing literature on cSLE around the world. Fortunately, 5-and 10-year mortality is lower than in adult-onset SLE, and patient and renal survival has improved over the years. However, relapses are common, especially during the transition from adolescence to adulthood. Patients with cSLE also accrue organ damage and co-morbid conditions from lupus and their therapies, including cardiovascular, infectious, ocular, skeletal, growth, endocrine, and reproductive complications. © Springer Nature Switzerland AG 2009, 2016, 2022.engrestrictedAccessAutoantibodiesBiopsyComplementLupus nephritisNephrotic syndromeSystemic lupus erythematosusbookPartCopyright SPRINGER INTERNATIONAL PUBLISHING10.1007/978-3-030-52719-8_97