title: Renal involvement in hypocomplementaemic urticarial vasculitis syndrome: a report of three paediatric cases.
abstract: OBJECTIVE
  To describe the diagnosis and management of renal disease in three paediatric cases of hypocomplementaemic urticarial vasculitis syndrome (HUVS).
  
  
  METHODS
  Three children who were diagnosed with HUVS and developed abnormalities of renal function during the disease course are described.
  
  
  RESULTS
  Urinary findings were heterogeneous: all the patients developed persistent microhaematuria, which was isolated in patient 1, associated with mild proteinuria in patient 2 and with nephrotic syndrome in patient 3. Renal biopsies were performed in all the patients: patients 1 and 2, who had normal levels of serum autoantibodies, shared a full-house IF (C3, C1q and Ig deposits), compatible with an SLE-like disease; patient 3 showed negative staining for IgG and IgM, but developed positive anti-dsDNA without fulfilling criteria for the diagnosis of SLE.
  
  
  CONCLUSIONS
  Renal involvement in HUVS is probably more frequent and more severe than in adults and may appear later. Isolated microhaematuria can be the only sign of subclinical renal involvement: its role should not be underestimated and a renal biopsy should be considered. Previous observations of rapidly progressive nephritis and consequent end-stage renal disease in children suggest the need for prompt diagnosis and treatment of renal involvement.
authors.affiliation: Department of Pediatrics, Azienda Ospedaliero-Universitaria Sant'Orsola-Malpighi, Bologna, Department of Pediatric Medicine, IRCCS Ospedale Pediatrico Bambin Gesù, Roma and Department of Pediatrics, Ospedale degli Infermi, Rimini, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Pasini
authors.affiliation: Department of Pediatrics, Azienda Ospedaliero-Universitaria Sant'Orsola-Malpighi, Bologna, Department of Pediatric Medicine, IRCCS Ospedale Pediatrico Bambin Gesù, Roma and Department of Pediatrics, Ospedale degli Infermi, Rimini, Italy.
authors.forename: Claudia
authors.initials: C
authors.lastname: Bracaglia
authors.affiliation: Department of Pediatrics, Azienda Ospedaliero-Universitaria Sant'Orsola-Malpighi, Bologna, Department of Pediatric Medicine, IRCCS Ospedale Pediatrico Bambin Gesù, Roma and Department of Pediatrics, Ospedale degli Infermi, Rimini, Italy. arianna.aceti2@unibo.it.
authors.forename: Arianna
authors.initials: A
authors.lastname: Aceti
authors.affiliation: Department of Pediatrics, Azienda Ospedaliero-Universitaria Sant'Orsola-Malpighi, Bologna, Department of Pediatric Medicine, IRCCS Ospedale Pediatrico Bambin Gesù, Roma and Department of Pediatrics, Ospedale degli Infermi, Rimini, Italy.
authors.forename: Marina
authors.initials: M
authors.lastname: Vivarelli
authors.affiliation: Department of Pediatrics, Azienda Ospedaliero-Universitaria Sant'Orsola-Malpighi, Bologna, Department of Pediatric Medicine, IRCCS Ospedale Pediatrico Bambin Gesù, Roma and Department of Pediatrics, Ospedale degli Infermi, Rimini, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Lavacchini
authors.affiliation: Department of Pediatrics, Azienda Ospedaliero-Universitaria Sant'Orsola-Malpighi, Bologna, Department of Pediatric Medicine, IRCCS Ospedale Pediatrico Bambin Gesù, Roma and Department of Pediatrics, Ospedale degli Infermi, Rimini, Italy.
authors.forename: Angela
authors.initials: A
authors.lastname: Miniaci
authors.affiliation: Department of Pediatrics, Azienda Ospedaliero-Universitaria Sant'Orsola-Malpighi, Bologna, Department of Pediatric Medicine, IRCCS Ospedale Pediatrico Bambin Gesù, Roma and Department of Pediatrics, Ospedale degli Infermi, Rimini, Italy.
authors.forename: Fabrizio
authors.initials: F
authors.lastname: De Benedetti
authors.affiliation: Department of Pediatrics, Azienda Ospedaliero-Universitaria Sant'Orsola-Malpighi, Bologna, Department of Pediatric Medicine, IRCCS Ospedale Pediatrico Bambin Gesù, Roma and Department of Pediatrics, Ospedale degli Infermi, Rimini, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Montini
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D009404:Nephrotic Syndrome / Q000175:diagnosis* / Q000276:immunology; D011507:Proteinuria / Q000175:diagnosis* / Q000276:immunology; D013577:Syndrome; D014581:Urticaria / Q000175:diagnosis* / Q000276:immunology; D014657:Vasculitis / Q000175:diagnosis* / Q000276:immunology
keywords: children; hypocomplementemic urticarial vasculitis syndrome; renal involvement