title: The indications, efficacy and adverse events of rituximab in a large cohort of patients with juvenile-onset SLE.
abstract: BACKGROUND
  B cells drive antibody formation and T cell activation. This study aimed to describe the clinical indications, efficacy and adverse events (AEs) for the B-cell depleting agent, rituximab, in a large cohort of children with lupus.
  
  
  METHODS
  Prescribing records and the UK JSLE Cohort Study database identified rituximab use.
  
  
  RESULTS
  Sixty-three patients received 104 courses of intravenous rituximab over a 10-year period. Patients were aged 12.2 (IQR 9.0-13.9) years at diagnosis and 50 (79%) were female. They had disease for 1.4 (0.2-3.0) years at the time of rituximab. Lupus nephritis was the most common indication (36% of first courses). Clinical biomarkers, 2.5 (1.6-4.3) months after treatment, demonstrated a statistically significant improvement in ESR, C3, C4, creatinine, albumin, haemoglobin, anti-dsDNA titres and urine albumin:creatinine ratio. IgG, IgA and IgM levels decreased (p < 0.01). Oral corticosteroid dose significantly reduced after rituximab (dose before 0.26 (0.09-0.44) mg/kg, after 0.17 (0.09-0.30) mg/kg; p = 0.01)). AEs occurred in 19 (18%) of all courses including; delayed second dose (8%), Ig replacement (2%) and infusion reactions (6%; anaphylaxis 2%). The global BILAG score showed a trend toward improvement (before 4.5 (2.0-9.0), after 3.0 (2.0-5.0); p = 0.16).
  
  
  CONCLUSIONS
  Rituximab improves disease activity in children with lupus and serious AEs are infrequent. Controlled studies are required.
authors.affiliation: Department of Women's and Children's Health, Institute of Translational Medicine, University of Liverpool, UK Department of Paediatric Nephrology, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK louise.watson@liverpool.ac.uk.
authors.forename: L
authors.initials: L
authors.lastname: Watson
authors.affiliation: Department of Women's and Children's Health, Institute of Translational Medicine, University of Liverpool, UK Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK.
authors.forename: M W
authors.initials: MW
authors.lastname: Beresford
authors.affiliation: Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust, London UK.
authors.forename: C
authors.initials: C
authors.lastname: Maynes
authors.affiliation: Department of Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Foundation Trust, London UK.
authors.forename: C
authors.initials: C
authors.lastname: Pilkington
authors.affiliation: Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust, London UK.
authors.forename: S D
authors.initials: SD
authors.lastname: Marks
authors.affiliation: Department of Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Foundation Trust, London UK.
authors.forename: Y
authors.initials: Y
authors.lastname: Glackin
authors.affiliation: Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust, London UK.
authors.forename: K
authors.initials: K
authors.lastname: Tullus
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000419:Albuminuria / Q000652:urine; D000974:Antibodies, Antinuclear / Q000097:blood; D058846:Antibodies, Monoclonal, Murine-Derived / Q000009:adverse effects / Q000627:therapeutic use*; D001402:B-Lymphocytes; D015415:Biomarkers / Q000097:blood / Q000652:urine; D001799:Blood Sedimentation; D002648:Child; D003176:Complement C3 / Q000378:metabolism; D003181:Complement C4 / Q000378:metabolism; D003404:Creatinine / Q000097:blood / Q000652:urine; D004247:DNA / Q000276:immunology; D005260:Female; D006454:Hemoglobins / Q000378:metabolism; D006801:Humans; D007136:Immunoglobulins / Q000097:blood; D007155:Immunologic Factors / Q000009:adverse effects / Q000627:therapeutic use*; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000188:drug therapy*; D018655:Lymphocyte Count; D008297:Male; D012189:Retrospective Studies; D000069283:Rituximab; D012709:Serum Albumin / Q000378:metabolism
chemical_list: D000305:Adrenal Cortex Hormones; D000974:Antibodies, Antinuclear; D058846:Antibodies, Monoclonal, Murine-Derived; D015415:Biomarkers; D003176:Complement C3; D003181:Complement C4; D006454:Hemoglobins; D007136:Immunoglobulins; D007155:Immunologic Factors; D012709:Serum Albumin; D000069283:Rituximab; D004247:DNA; D003404:Creatinine
keywords: B-cell depletion; Rituximab; SLE; child; lupus