Chapter 28
ANCA-associated Vasculitis
Book Editor(s):Jonathan C. Craig MBChB, DipCH, MMed(Clin Epi), PhD, FAHMS,
Donald A. Molony MD,
Giovanni F.M. Strippoli MD, PhD, MPH, MM (Epi),
Jonathan C. Craig MBChB, DipCH, MMed(Clin Epi), PhD, FAHMS
Matthew Flinders Distinguished Professor Vice President and Executive Dean
College of Medicine and Public Health, Flinders University, Adelaide, Australia
Search for more papers by this authorDonald A. Molony MD
Professor of Medicine Distinguished Teaching Professor of the University of Texas System
Division of Renal Diseases and Hypertension AND Center for Clinical Research and Evidence-based Medicine, McGovern Medical School University of Texas, Houston, TX, USA
Search for more papers by this authorGiovanni F.M. Strippoli MD, PhD, MPH, MM (Epi)
Professor of Nephrology Adjunct Professor of Epidemiology
Department of Emergency and Organ Transplantation – University of Bari, Bari, Italy
School of Public Health, University of Sydney, Sydney, NSW, Australia
Search for more papers by this authorFirst published: 18 November 2022
Summary
The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are a group of systemic necrotizing vasculitides which affect small to medium-sized blood vessels. There is a genetic basis for AAV, and this may underlie some of the reported population differences. There are also several reported environmental associations with AAV such as toxins, drugs, and infections. AAV is a multisystem disease and clinical presentation varies widely in both severity and organ involvement. Several clinical trials have studied the optimum route, dose, and duration of treatment with cyclophosphamide. Several studies have compared other induction agents to cyclophosphamide in AAV and these could be considered for resistant disease but are not currently recommended for severe disease in published guidelines. Patients with minor relapses may respond to an increase or reintroduction of their oral immunosuppression or steroids.
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