title: Validation of the Lupus Nephritis Clinical Indices in Childhood-Onset Systemic Lupus Erythematosus.
abstract: OBJECTIVE
  To validate clinical indices of lupus nephritis activity and damage when used in children against the criterion standard of kidney biopsy findings.
  
  
  METHODS
  In 83 children requiring kidney biopsy, the Systemic Lupus Erythematosus Disease Activity Index renal domain (SLEDAI-R), British Isles Lupus Assessment Group index renal domain (BILAG-R), Systemic Lupus International Collaborating Clinics (SLICC) renal activity score (SLICC-RAS), and SLICC Damage Index renal domain (SDI-R) were measured. Fixed effects and logistic models were calculated to predict International Society of Nephrology/Renal Pathology Society (ISN/RPS) class; low-to-moderate versus high lupus nephritis activity (National Institutes of Health [NIH] activity index [AI]) score: ≤10 versus >10; tubulointerstitial activity index (TIAI) score: ≤5 versus >5; or the absence versus presence of lupus nephritis chronicity (NIH chronicity index) score: 0 versus ≥1.
  
  
  RESULTS
  There were 10, 50, and 23 patients with ISN/RPS class I/II, III/IV, and V, respectively. Scores of the clinical indices did not differentiate among patients by ISN/RPS class. The SLEDAI-R and SLICC-RAS but not the BILAG-R differed with lupus nephritis activity status defined by NIH-AI scores, while only the SLEDAI-R scores differed between lupus nephritis activity status based on TIAI scores. The sensitivity and specificity of the SDI-R to capture lupus nephritis chronicity was 23.5% and 91.7%, respectively. Despite being designed to measure lupus nephritis activity, SLICC-RAS and SLEDAI-R scores significantly differed with lupus nephritis chronicity status.
  
  
  CONCLUSIONS
  Current clinical indices of lupus nephritis fail to discriminate ISN/RPS class in children. Despite its shortcomings, the SLEDAI-R appears best for measuring lupus nephritis activity in a clinical setting. The SDI-R is a poor correlate of lupus nephritis chronicity.
authors.affiliation: Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
authors.forename: Rina
authors.initials: R
authors.lastname: Mina
authors.affiliation: Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
authors.forename: Khalid
authors.initials: K
authors.lastname: Abulaban
authors.affiliation: Ann & Lurie Children's Hospital, Chicago, Illinois.
authors.forename: Marisa S
authors.initials: MS
authors.lastname: Klein-Gitelman
authors.affiliation: Steven and Alexandra Cohen Children's Medical Center of New York, New York.
authors.forename: Barbara A
authors.initials: BA
authors.lastname: Eberhard
authors.affiliation: Nationwide Children's Hospital and Ohio State University, Columbus.
authors.forename: Stacy P
authors.initials: SP
authors.lastname: Ardoin
authors.affiliation: MetroHealth Medical Center and Case Western Reserve University, Cleveland, Ohio.
authors.forename: Nora
authors.initials: N
authors.lastname: Singer
authors.affiliation: University of Chicago Comer Children's Hospital, Chicago, Illinois.
authors.forename: Karen
authors.initials: K
authors.lastname: Onel
authors.affiliation: British Columbia Children's Hospital, Vancouver, British Columbia, Canada.
authors.forename: Lori
authors.initials: L
authors.lastname: Tucker
authors.affiliation: Riley Hospital for Children, Indianapolis, Indiana.
authors.forename: Kathleen
authors.initials: K
authors.lastname: O'neil
authors.affiliation: Texas Scottish Rite Hospital, Dallas.
authors.forename: Tracey
authors.initials: T
authors.lastname: Wright
authors.affiliation: University Hospitals Rainbow Babies & Children's Hospital, Cleveland, Ohio.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Brooks
authors.affiliation: Children's Healthcare of Atlanta, Atlanta, Georgia.
authors.forename: Kelly
authors.initials: K
authors.lastname: Rouster-Stevens
authors.affiliation: Children's National Health System, Washington, DC.
authors.forename: Lawrence
authors.initials: L
authors.lastname: Jung
authors.affiliation: Columbia University Medical Center, New York, New York.
authors.forename: Lisa
authors.initials: L
authors.lastname: Imundo
authors.affiliation: Ohio State University Wexner Medical Center, Columbus.
authors.forename: Brad
authors.initials: B
authors.lastname: Rovin
authors.affiliation: Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
authors.forename: David
authors.initials: D
authors.lastname: Witte
authors.affiliation: University of Cincinnati, Cincinnati, Ohio.
authors.forename: Jun
authors.initials: J
authors.lastname: Ying
authors.affiliation: Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
authors.forename: Hermine I
authors.initials: HI
authors.lastname: Brunner
mesh_terms: D000293:Adolescent; D005260:Female; D006801:Humans; D007668:Kidney / Q000473:pathology; D008181:Lupus Nephritis / Q000473:pathology; D008297:Male; D012720:Severity of Illness Index