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Antineutrophil cytoplasmic antibody-associated vasculitis, update on molecular pathogenesis, diagnosis, and treatment

Authors Arman F, Barsoum M, Selamet U, Shakeri H, Wassef O, Mikhail M, Rastogi A, Hanna RM

Received 9 September 2018

Accepted for publication 24 October 2018

Published 22 November 2018 Volume 2018:11 Pages 313—319


Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 2

Editor who approved publication: Professor Pravin Singhal

Farid Arman,1 Marina Barsoum,1 Umut Selamet,1 Hania Shakeri,1 Olivia Wassef,1 Mira Mikhail,2 Anjay Rastogi,1 Ramy M Hanna1

1Department of Medicine, Division of Nephrology, UCLA David Geffen School of Medicine, Los Angeles, CA, USA; 2College of Biological Sciences, Biola University, La Mirada, CA, USA

Abstract: Circulating antineutrophil cytoplasmic antibodies (ANCAs) are the central pathogenic mechanism for a group of systemic and renal syndromes called the ANCA-associated vasculitis (AAV). The nomenclature has changed from eponymous labeling to granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis. These syndromes predominantly affect the pulmonary and renal systems. We also review the molecular pathology behind ANCAs and associated antigens and infections. Various clinical presentations, the multiple target organs affected, and diagnostic challenges involved in identifying these diseases are discussed. Treatment updates are also provided with regard to new studies and the now standard use of anti-CD-20 monoclonal antibodies as first-line therapy in all but the most aggressive presentations of this disease. Maintenance regimens and monitoring strategies for relapse of vasculitis and associated systemic complications are discussed.

Keywords: proteinuria, glomerulonephritis, ANCA associated vasculitis, rituximab, P-ANCA, C-ANCA

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