Chinar Rahmattulla1, Robert A F de Lind van Wijngaarden2, Annelies E Berden2, Herbert A Hauer2, Oliver Floßmann2, David R W Jayne2, Gill Gaskin2, Niels Rasmussen2, Laure-Hélène Noël2, Franco Ferrario2, Rüdiger Waldherr2, Ron Wolterbeek2, Arda Göçeroğlu2, Charles D Pusey2, E Christiaan Hagen2, Jan A Bruijn2, Ingeborg M Bajema2. 1. 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. 2. 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.
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. CONCLUSION: 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.
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. CONCLUSION: 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.