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

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

Research output: Contribution to journalReview articlepeer-review

8 Scopus citations

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.

Original languageEnglish
Pages (from-to)313-319
Number of pages7
JournalInternational Journal of Nephrology and Renovascular Disease
Volume11
DOIs
StatePublished - 2018
Externally publishedYes

Keywords

  • ANCA associated vasculitis
  • C-ANCA
  • Glomerulonephritis
  • P-ANCA
  • Proteinuria
  • Rituximab

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