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Vasculitis working group: Selected unanswered questions related to giant cell arteritis and anti-neutrophil cytoplasmic antibody-associated vasculitis
Authors:Steven R Ytterberg  Kenneth J Warrington
Institution:1. Department of Neurology, Mount Sinai Beth Israel, and Department of Neurology, Icahn School of Medicine, Mount Sinai, New York, NY, USA;2. Department of Neurology, Albert Einstein College of Medicine, Montefiore Medical Center, Bronx, NY, USA;3. Department of Neurology, University of Virginia Health System, Charlottesville, VA, USA;4. Division of Human Genetics, Cincinnati Children''s Hospital Medical Center, and Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA;5. Department of Neurology, Massachusetts General Hospital, Boston, MA, USA;6. Department of Neurology and Psychiatry, Taub Institute, and Sergievsky Center, Columbia University, College of Physicians and Surgeons, New York, NY, USA;7. Movement Disorders Unit, Neurological Institute, Tel Aviv Medical Center, Sackler School of Medicine, Sagol School of Neuroscience, Tel-Aviv University, Tel Aviv, Israel;1. School of Geography, Planning and Environmental Policy, University College Dublin, Newman Building, Belfield, Dublin 4, Ireland;2. Department of Geography and Earth Sciences, Aberystwyth University, Llandinam Building, Penglais Campus, Aberystwyth, SY23 3DB, UK;3. Innovative River Solutions, Institute of Agriculture and Environment, Massey University, Private Bag 11 222, Palmerston North 4442, New Zealand;4. Museo Nacional de Ciencias Naturales-CSIC, Serrano 115bis, 28006 Madrid, Spain;1. Laboratori d’Enginyeria Marítima, Universitat Politècnica de Catalunya, C/ Jordi Girona, 1-3 Ed. D1 Campus Nord, 08034 Barcelona, Spain;2. Jordi Girona, 1–3, Ed. D1, 08031 Barcelona, Spain;3. MaREI Centre, Environmental Research Institute, University College Cork, Ringaskiddy, Co. Cork, Ireland;4. Natl. Institute of Marine Geology and GeoEcology — GeoEcoMar, Str. Dimitrie Onciul 23-25, Sector 2, 024053 Bucharest, Romania;1. Management School, Universidad EAFIT, Carrera 49 N 7 sur – 50, Medellín 0–50022, Colombia;2. Victoria Business School, Victoria University of Wellington, PO Box 600, Wellington, New Zealand;3. Centre for Systems Studies, Business School, University of Hull, Hull HU6 7RX, United Kingdom;4. Victoria Business School, Victoria University of Wellington, PO Box 600, Wellington, New Zealand;5. School of Innovation, Design and Engineering, Mälardalen University, Sweden;6. School of Political and Social Sciences, University of Canterbury, New Zealand;7. School of Agriculture and Food Sciences, University of Queensland, Australia
Abstract:Evidence to guide assessment and management of patients with vasculitis is lacking for many important clinical questions. The evidence surrounding several common questions about management of vasculitis was reviewed. Patients with giant cell arteritis (GCA) are at risk for developing extra-cranial large vessel inflammation. Clinicians should be aware of this complication and search for large vessel involvement in patients with GCA who have ischemic symptoms. Research is needed to define optimal strategies to identify patients with such complications. Because of the hazards of chronic corticosteroid use, alternative therapies for patients with GCA have been sought but thus far no clear alternatives have been identified. Anti-neutrophil cytoplasmic antibodies (ANCA) are associated with small-vessel vasculitis, including Wegener's granulomatosis and microscopic polyangiitis, but changes in ANCA titers should not be used as a surrogate biomarker for disease activity. Several immunosuppressive agents can be used for maintenance therapy after induction of remission in patients with ANCA-associated vasculitis, with no firm evidence that one agent is superior to others. Collectively, this review shows that more research is needed to provide a firmer body of evidence to support clinical decision-making for patients with vasculitis.
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