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Review
. 2018 Nov 22:11:313-319.
doi: 10.2147/IJNRD.S162071. eCollection 2018.

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

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

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

Farid Arman et al. Int J Nephrol Renovasc Dis. .

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: ANCA associated vasculitis; C-ANCA; P-ANCA; glomerulonephritis; proteinuria; rituximab.

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Conflict of interest statement

Disclosure The authors report no conflicts of interest in this work.

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