title: Renal function and ear, nose, throat involvement in anti-neutrophil cytoplasmic antibody-associated vasculitis: prospective data from the European Vasculitis Society clinical trials.
abstract: OBJECTIVE
  We investigated whether ENT involvement is associated with renal biopsy findings and renal function in patients with ANCA-associated vasculitis (AAV).
  
  
  METHODS
  Newly diagnosed AAV patients derived from three international, multicentre trials were included. To investigate an association between ENT involvement and estimated glomerular filtration rate (eGFR) at diagnosis and 5-year follow-up, we performed multivariable regression analyses including clinical and histopathological parameters. To investigate whether our findings are specific to ENT involvement, we performed comparable analyses between eGFR and other early disease manifestations (arthralgia/arthritis, cutaneous and lung involvement).
  
  
  RESULTS
  One hundred and eighty-five of the 414 patients had ENT involvement. The mean presenting eGFR of patients with and without ENT involvement was 39.16 and 23.88 ml/min/1.73 m(2), respectively (P < 0.001). Mean eGFR increased by 6.76 ml/min/1.73 m(2) with each added ENT symptom (P = 0.007). Patients with ENT involvement had less interstitial fibrosis and tubular atrophy and a prognostically more favourable histopathological class on renal biopsy examination. Multivariable regression analyses correcting for clinical and histopathological parameters showed that ENT involvement is associated with both baseline and 5-year follow-up eGFR. There were no associations between baseline and 5-year follow-up eGFR and arthralgia/arthritis, cutaneous or lung involvement, suggesting that our findings are specific to ENT involvement.
  
  
  CONCLUSIONS
  The presence of ENT involvement in AAV patients is associated with prognostically favourable renal biopsy findings and better renal function. These results indicate that there may be different phenotypes of AAV defined by ENT involvement.
authors.affiliation: Department of Pathology, Leiden University Medical Center, Leiden, Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Cardiology Centers of The Netherlands, Amsterdam, The Netherlands, Renal Unit, Royal Berkshire Hospital, Reading, Department of Medicine, University of Cambridge, Department of Medicine, Hammersmith Hospital, Imperial College London, London, UK, Department of Biochemistry and Immunology, Statens Seruminstitut, Copenhagen, Denmark, Department of Pathology, Necker Hospital, René Descartes University, Paris, France, Nephropathology Center, San Gerardo Hospital, Monza, Italy, Department of Pathology, University of Heidelberg, Heidelberg, Germany, Deparment of Medical Statistics & Bioinformatics, Leiden University Medical Center, Leiden and Department of Nephrology, Meander Medical Center, Amersfoort, The Netherlands c.rahmattulla@lumc.nl.
authors.forename: Chinar
authors.initials: C
authors.lastname: Rahmattulla
authors.affiliation: Department of Pathology, Leiden University Medical Center, Leiden, Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Cardiology Centers of The Netherlands, Amsterdam, The Netherlands, Renal Unit, Royal Berkshire Hospital, Reading, Department of Medicine, University of Cambridge, Department of Medicine, Hammersmith Hospital, Imperial College London, London, UK, Department of Biochemistry and Immunology, Statens Seruminstitut, Copenhagen, Denmark, Department of Pathology, Necker Hospital, René Descartes University, Paris, France, Nephropathology Center, San Gerardo Hospital, Monza, Italy, Department of Pathology, University of Heidelberg, Heidelberg, Germany, Deparment of Medical Statistics & Bioinformatics, Leiden University Medical Center, Leiden and Department of Nephrology, Meander Medical Center, Amersfoort, The Netherlands.
authors.forename: Robert A F
authors.initials: RA
authors.lastname: de Lind van Wijngaarden
authors.affiliation: Department of Pathology, Leiden University Medical Center, Leiden, Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Cardiology Centers of The Netherlands, Amsterdam, The Netherlands, Renal Unit, Royal Berkshire Hospital, Reading, Department of Medicine, University of Cambridge, Department of Medicine, Hammersmith Hospital, Imperial College London, London, UK, Department of Biochemistry and Immunology, Statens Seruminstitut, Copenhagen, Denmark, Department of Pathology, Necker Hospital, René Descartes University, Paris, France, Nephropathology Center, San Gerardo Hospital, Monza, Italy, Department of Pathology, University of Heidelberg, Heidelberg, Germany, Deparment of Medical Statistics & Bioinformatics, Leiden University Medical Center, Leiden and Department of Nephrology, Meander Medical Center, Amersfoort, The Netherlands.
authors.forename: Annelies E
authors.initials: AE
authors.lastname: Berden
authors.affiliation: Department of Pathology, Leiden University Medical Center, Leiden, Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Cardiology Centers of The Netherlands, Amsterdam, The Netherlands, Renal Unit, Royal Berkshire Hospital, Reading, Department of Medicine, University of Cambridge, Department of Medicine, Hammersmith Hospital, Imperial College London, London, UK, Department of Biochemistry and Immunology, Statens Seruminstitut, Copenhagen, Denmark, Department of Pathology, Necker Hospital, René Descartes University, Paris, France, Nephropathology Center, San Gerardo Hospital, Monza, Italy, Department of Pathology, University of Heidelberg, Heidelberg, Germany, Deparment of Medical Statistics & Bioinformatics, Leiden University Medical Center, Leiden and Department of Nephrology, Meander Medical Center, Amersfoort, The Netherlands.
authors.forename: Herbert A
authors.initials: HA
authors.lastname: Hauer
authors.affiliation: Department of Pathology, Leiden University Medical Center, Leiden, Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Cardiology Centers of The Netherlands, Amsterdam, The Netherlands, Renal Unit, Royal Berkshire Hospital, Reading, Department of Medicine, University of Cambridge, Department of Medicine, Hammersmith Hospital, Imperial College London, London, UK, Department of Biochemistry and Immunology, Statens Seruminstitut, Copenhagen, Denmark, Department of Pathology, Necker Hospital, René Descartes University, Paris, France, Nephropathology Center, San Gerardo Hospital, Monza, Italy, Department of Pathology, University of Heidelberg, Heidelberg, Germany, Deparment of Medical Statistics & Bioinformatics, Leiden University Medical Center, Leiden and Department of Nephrology, Meander Medical Center, Amersfoort, The Netherlands.
authors.forename: Oliver
authors.initials: O
authors.lastname: Floßmann
authors.affiliation: Department of Pathology, Leiden University Medical Center, Leiden, Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Cardiology Centers of The Netherlands, Amsterdam, The Netherlands, Renal Unit, Royal Berkshire Hospital, Reading, Department of Medicine, University of Cambridge, Department of Medicine, Hammersmith Hospital, Imperial College London, London, UK, Department of Biochemistry and Immunology, Statens Seruminstitut, Copenhagen, Denmark, Department of Pathology, Necker Hospital, René Descartes University, Paris, France, Nephropathology Center, San Gerardo Hospital, Monza, Italy, Department of Pathology, University of Heidelberg, Heidelberg, Germany, Deparment of Medical Statistics & Bioinformatics, Leiden University Medical Center, Leiden and Department of Nephrology, Meander Medical Center, Amersfoort, The Netherlands.
authors.forename: David R W
authors.initials: DR
authors.lastname: Jayne
authors.affiliation: Department of Pathology, Leiden University Medical Center, Leiden, Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Cardiology Centers of The Netherlands, Amsterdam, The Netherlands, Renal Unit, Royal Berkshire Hospital, Reading, Department of Medicine, University of Cambridge, Department of Medicine, Hammersmith Hospital, Imperial College London, London, UK, Department of Biochemistry and Immunology, Statens Seruminstitut, Copenhagen, Denmark, Department of Pathology, Necker Hospital, René Descartes University, Paris, France, Nephropathology Center, San Gerardo Hospital, Monza, Italy, Department of Pathology, University of Heidelberg, Heidelberg, Germany, Deparment of Medical Statistics & Bioinformatics, Leiden University Medical Center, Leiden and Department of Nephrology, Meander Medical Center, Amersfoort, The Netherlands.
authors.forename: Gill
authors.initials: G
authors.lastname: Gaskin
authors.affiliation: Department of Pathology, Leiden University Medical Center, Leiden, Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Cardiology Centers of The Netherlands, Amsterdam, The Netherlands, Renal Unit, Royal Berkshire Hospital, Reading, Department of Medicine, University of Cambridge, Department of Medicine, Hammersmith Hospital, Imperial College London, London, UK, Department of Biochemistry and Immunology, Statens Seruminstitut, Copenhagen, Denmark, Department of Pathology, Necker Hospital, René Descartes University, Paris, France, Nephropathology Center, San Gerardo Hospital, Monza, Italy, Department of Pathology, University of Heidelberg, Heidelberg, Germany, Deparment of Medical Statistics & Bioinformatics, Leiden University Medical Center, Leiden and Department of Nephrology, Meander Medical Center, Amersfoort, The Netherlands.
authors.forename: Niels
authors.initials: N
authors.lastname: Rasmussen
authors.affiliation: Department of Pathology, Leiden University Medical Center, Leiden, Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Cardiology Centers of The Netherlands, Amsterdam, The Netherlands, Renal Unit, Royal Berkshire Hospital, Reading, Department of Medicine, University of Cambridge, Department of Medicine, Hammersmith Hospital, Imperial College London, London, UK, Department of Biochemistry and Immunology, Statens Seruminstitut, Copenhagen, Denmark, Department of Pathology, Necker Hospital, René Descartes University, Paris, France, Nephropathology Center, San Gerardo Hospital, Monza, Italy, Department of Pathology, University of Heidelberg, Heidelberg, Germany, Deparment of Medical Statistics & Bioinformatics, Leiden University Medical Center, Leiden and Department of Nephrology, Meander Medical Center, Amersfoort, The Netherlands.
authors.forename: Laure-Hélène
authors.initials: LH
authors.lastname: Noël
authors.affiliation: Department of Pathology, Leiden University Medical Center, Leiden, Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Cardiology Centers of The Netherlands, Amsterdam, The Netherlands, Renal Unit, Royal Berkshire Hospital, Reading, Department of Medicine, University of Cambridge, Department of Medicine, Hammersmith Hospital, Imperial College London, London, UK, Department of Biochemistry and Immunology, Statens Seruminstitut, Copenhagen, Denmark, Department of Pathology, Necker Hospital, René Descartes University, Paris, France, Nephropathology Center, San Gerardo Hospital, Monza, Italy, Department of Pathology, University of Heidelberg, Heidelberg, Germany, Deparment of Medical Statistics & Bioinformatics, Leiden University Medical Center, Leiden and Department of Nephrology, Meander Medical Center, Amersfoort, The Netherlands.
authors.forename: Franco
authors.initials: F
authors.lastname: Ferrario
authors.affiliation: Department of Pathology, Leiden University Medical Center, Leiden, Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Cardiology Centers of The Netherlands, Amsterdam, The Netherlands, Renal Unit, Royal Berkshire Hospital, Reading, Department of Medicine, University of Cambridge, Department of Medicine, Hammersmith Hospital, Imperial College London, London, UK, Department of Biochemistry and Immunology, Statens Seruminstitut, Copenhagen, Denmark, Department of Pathology, Necker Hospital, René Descartes University, Paris, France, Nephropathology Center, San Gerardo Hospital, Monza, Italy, Department of Pathology, University of Heidelberg, Heidelberg, Germany, Deparment of Medical Statistics & Bioinformatics, Leiden University Medical Center, Leiden and Department of Nephrology, Meander Medical Center, Amersfoort, The Netherlands.
authors.forename: Rüdiger
authors.initials: R
authors.lastname: Waldherr
authors.affiliation: Department of Pathology, Leiden University Medical Center, Leiden, Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Cardiology Centers of The Netherlands, Amsterdam, The Netherlands, Renal Unit, Royal Berkshire Hospital, Reading, Department of Medicine, University of Cambridge, Department of Medicine, Hammersmith Hospital, Imperial College London, London, UK, Department of Biochemistry and Immunology, Statens Seruminstitut, Copenhagen, Denmark, Department of Pathology, Necker Hospital, René Descartes University, Paris, France, Nephropathology Center, San Gerardo Hospital, Monza, Italy, Department of Pathology, University of Heidelberg, Heidelberg, Germany, Deparment of Medical Statistics & Bioinformatics, Leiden University Medical Center, Leiden and Department of Nephrology, Meander Medical Center, Amersfoort, The Netherlands.
authors.forename: Ron
authors.initials: R
authors.lastname: Wolterbeek
authors.affiliation: Department of Pathology, Leiden University Medical Center, Leiden, Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Cardiology Centers of The Netherlands, Amsterdam, The Netherlands, Renal Unit, Royal Berkshire Hospital, Reading, Department of Medicine, University of Cambridge, Department of Medicine, Hammersmith Hospital, Imperial College London, London, UK, Department of Biochemistry and Immunology, Statens Seruminstitut, Copenhagen, Denmark, Department of Pathology, Necker Hospital, René Descartes University, Paris, France, Nephropathology Center, San Gerardo Hospital, Monza, Italy, Department of Pathology, University of Heidelberg, Heidelberg, Germany, Deparment of Medical Statistics & Bioinformatics, Leiden University Medical Center, Leiden and Department of Nephrology, Meander Medical Center, Amersfoort, The Netherlands.
authors.forename: Arda
authors.initials: A
authors.lastname: Göçeroğlu
authors.affiliation: Department of Pathology, Leiden University Medical Center, Leiden, Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Cardiology Centers of The Netherlands, Amsterdam, The Netherlands, Renal Unit, Royal Berkshire Hospital, Reading, Department of Medicine, University of Cambridge, Department of Medicine, Hammersmith Hospital, Imperial College London, London, UK, Department of Biochemistry and Immunology, Statens Seruminstitut, Copenhagen, Denmark, Department of Pathology, Necker Hospital, René Descartes University, Paris, France, Nephropathology Center, San Gerardo Hospital, Monza, Italy, Department of Pathology, University of Heidelberg, Heidelberg, Germany, Deparment of Medical Statistics & Bioinformatics, Leiden University Medical Center, Leiden and Department of Nephrology, Meander Medical Center, Amersfoort, The Netherlands.
authors.forename: Charles D
authors.initials: CD
authors.lastname: Pusey
authors.affiliation: Department of Pathology, Leiden University Medical Center, Leiden, Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Cardiology Centers of The Netherlands, Amsterdam, The Netherlands, Renal Unit, Royal Berkshire Hospital, Reading, Department of Medicine, University of Cambridge, Department of Medicine, Hammersmith Hospital, Imperial College London, London, UK, Department of Biochemistry and Immunology, Statens Seruminstitut, Copenhagen, Denmark, Department of Pathology, Necker Hospital, René Descartes University, Paris, France, Nephropathology Center, San Gerardo Hospital, Monza, Italy, Department of Pathology, University of Heidelberg, Heidelberg, Germany, Deparment of Medical Statistics & Bioinformatics, Leiden University Medical Center, Leiden and Department of Nephrology, Meander Medical Center, Amersfoort, The Netherlands.
authors.forename: E Christiaan
authors.initials: EC
authors.lastname: Hagen
authors.affiliation: Department of Pathology, Leiden University Medical Center, Leiden, Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Cardiology Centers of The Netherlands, Amsterdam, The Netherlands, Renal Unit, Royal Berkshire Hospital, Reading, Department of Medicine, University of Cambridge, Department of Medicine, Hammersmith Hospital, Imperial College London, London, UK, Department of Biochemistry and Immunology, Statens Seruminstitut, Copenhagen, Denmark, Department of Pathology, Necker Hospital, René Descartes University, Paris, France, Nephropathology Center, San Gerardo Hospital, Monza, Italy, Department of Pathology, University of Heidelberg, Heidelberg, Germany, Deparment of Medical Statistics & Bioinformatics, Leiden University Medical Center, Leiden and Department of Nephrology, Meander Medical Center, Amersfoort, The Netherlands.
authors.forename: Jan A
authors.initials: JA
authors.lastname: Bruijn
authors.affiliation: Department of Pathology, Leiden University Medical Center, Leiden, Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, Cardiology Centers of The Netherlands, Amsterdam, The Netherlands, Renal Unit, Royal Berkshire Hospital, Reading, Department of Medicine, University of Cambridge, Department of Medicine, Hammersmith Hospital, Imperial College London, London, UK, Department of Biochemistry and Immunology, Statens Seruminstitut, Copenhagen, Denmark, Department of Pathology, Necker Hospital, René Descartes University, Paris, France, Nephropathology Center, San Gerardo Hospital, Monza, Italy, Department of Pathology, University of Heidelberg, Heidelberg, Germany, Deparment of Medical Statistics & Bioinformatics, Leiden University Medical Center, Leiden and Department of Nephrology, Meander Medical Center, Amersfoort, The Netherlands.
authors.forename: Ingeborg M
authors.initials: IM
authors.lastname: Bajema
mesh_terms: D000328:Adult; D000368:Aged; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000175:diagnosis* / Q000503:physiopathology*; D001706:Biopsy; D002986:Clinical Trials as Topic; D004423:Ear / Q000503:physiopathology*; D005060:Europe; D005260:Female; D005500:Follow-Up Studies; D005919:Glomerular Filtration Rate / Q000502:physiology; D006801:Humans; D007668:Kidney / Q000473:pathology / Q000503:physiopathology*; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D009666:Nose / Q000503:physiopathology*; D010614:Pharynx / Q000503:physiopathology*; D010641:Phenotype; D011379:Prognosis; D011446:Prospective Studies; D012044:Regression Analysis
keywords: ANCA; ENT; renal function; renal histology; vasculitis