Life-threatening extrarenal lupus in children despite improvement in serologic findings.
Systemic lupus erythematosus (SLE) is an autoimmune disorder with the potential for multiorgan involvement. Serologic tests are helpful in establishing the diagnosis of SLE and predicting disease flares. However, there are few data on the relationship between the onset of new organ involvement and lupus serologies, especially in children. This report details our experience in managing two children with lupus nephritis. Both developed life-threatening extrarenal complications (cerebritis and carditis) soon after receiving high-dose immunosuppressive therapy and despite normalizing serologies. This lack of concordance between serologies and the development of carditis and cerebritis needs to be recognized so that health care professionals treating children with SLE can promptly intensify immunosuppressive medications and avoid life-threatening delays from seeking alternative explanations for symptomatology.
Butani
Lavjay
L
Section of Pediatric Nephrology, University of California Davis, Ticon II, Third Floor, 2516 Stockton Blvd, Sacramento, CA 95817, USA. lavjay.butani@ucdmc.ucdavis.edu
Makker
Sudesh P
SP
D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D002648:Child; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000150:complications* / Q000188:drug therapy*; D008181:Lupus Nephritis / Q000188:drug therapy* / Q000209:etiology; D020945:Lupus Vasculitis, Central Nervous System / Q000188:drug therapy* / Q000209:etiology; D008775:Methylprednisolone / Q000627:therapeutic use*; D009205:Myocarditis / Q000188:drug therapy* / Q000209:etiology; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D008775:Methylprednisolone