[Childhood-onset systemic lupus]

CHILDHOOD-ONSET SYSTEMIC LUPUS. Childhood-onset systemic lupus (SL) is a rare multifactorial autoimmune disease belonging to connectivitis. Clinical presentation is very heterogeneous with multi-systemic involvement: skin, joint, renal, hematological, neuropsychiatric, pulmonary, cardiac among others. Renal involvement is particularly frequent and severe in children and must be checked ou on a regular basis by screening for proteinuria. Lab exams show the production of antibodies directed against the native double-stranded DNA, excessive production of type I interferon, consumption of complement is observed during periods of flare-up. There are rare forms of monogenic SL which must be evocated in case of early-onset, familial cases or when occurring in boys.