Severe paediatric systemic lupus erythematosus nephritis--a single-centre experience.
BACKGROUND
Paediatric patients with systemic lupus erythematosus (SLE) often have severe presentations including lupus nephritis (LN). Few paediatric studies have evaluated the anticardiolipin antibody (aCL) and renal histology. The purpose of this study was to evaluate clinicopathologic features, including aCL, short-term clinical and renal histologic outcomes of paediatric patients with new-onset SLE nephritis.


METHODS
We conducted a single centre, retrospective inception cohort study. Charts were reviewed at presentation (initial renal biopsy), 6-month (follow-up biopsy) and 12-month follow-up.


RESULTS
The population consisted of 21 patients (median age, 14.5 years): 19/21 were female, 6/21 African American, 3/21 Asian, 9/21 Caucasian and 3/21 Hispanic. At presentation, 19/21 had elevated aCL, 15/21 hypertensive, 12/21 nephrotic and 7/21 required haemodialysis (HD)-2/7 HD patients had thrombotic microangiopathy, 1/7 crescentic glomerulonephritis. Two patients had thromboembolism: both had aCL, were taking oral contraceptives and required HD, one was nephrotic and the other had elevated lupus anticoagulant. Initial biopsies revealed 6/21 ISN/RPS class II nephritis, 3/21 class III, 7/21 class IV and 5/21 class V. Treatment consisted of methylprednisolone, corticosteroids, cyclophosphamide or mycophenolate mofetil. Follow-up biopsies revealed 12/13 to have improved histology. Indication for a follow-up biopsy was severe illness at presentation. At 12-month follow-up, no patients were nephrotic (P < 0.001) or required HD (P < 0.001), and 3/14 had elevated aCL (P < 0.001).


CONCLUSIONS
Elevated aCL, hypertension, nephrotic syndrome and need for HD were common presentations among our paediatric SLE nephritis population. Renal histology and aCL were helpful in the therapeutic management.
Hobbs
David J
DJ
Pediatric Nephrology, Helen DeVos Children's Hospital and Michigan State University College of Human Medicine, Grand Rapids, MI, USA.
Barletta
Gina-Marie
GM
Rajpal
Jurat S
JS
Rajpal
Miriam N
MN
Weismantel
David P
DP
Birmingham
James D
JD
Bunchman
Timothy E
TE
D000293:Adolescent; D017153:Antibodies, Anticardiolipin / Q000097:blood; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008181:Lupus Nephritis / Q000097:blood / Q000150:complications / Q000175:diagnosis*; D008297:Male; D012189:Retrospective Studies; D012720:Severity of Illness Index; D055815:Young Adult
D017153:Antibodies, Anticardiolipin