ANCA-Associated Vasculitis

Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is a group of conditions characterized by the development of autoantibodies to the neutrophil proteins proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA), leading to systemic inflammation and damage to small blood vessels. AAV includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). Review our collection of articles on this topic.

FEATURED ARTICLE: Case Report: An Uncommon Presenting Complaint of GPA

Case Report: MPA Hiding in Plain Sight

Benjamin Aronow, MD, Eduardo Mantovani Cardoso, MD, Steffi Thomas, DO, Prashant Grover, MD, & Weishali Joshi, MD | May 12, 2022

The COVID-19 pandemic has filled hospitals with patients with rapidly progressive respiratory failure and diffuse bilateral opacities on chest X-ray.1 Additionally, many patients with severe COVID-19 develop acute kidney injury and require dialysis.2 Pulmonary-renal syndromes are also important to consider in this setting. Although alveolar hemorrhage is a cardinal feature of this syndrome, many patients…

How to Differentiate Between & Manage Hypereosinophilic Syndromes & EGPA

Samantha C. Shapiro, MD | February 2, 2022

Hypereosinophilic syndromes and eosinophilic granulomatosis with polyangiitis (EGPA) represent a spectrum of eosinophilic disorders. Amy D. Klion, MD, provided a detailed overview of this spectrum during ACR Convergence 2021.

Case Report: GPA Presenting as Neuropathy

Ann George, MD, Azin Azarfar, MD, Shazia Bég, MD, & Maria Farooq, MD | December 16, 2021

Granulomatosis with polyangiitis (GPA), is a type of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) that affects small- to medium-sized vessels.1 It can occur equally in both men and women, with a reported mean age at onset of 55 years.2 The classical presentation of GPA includes upper respiratory tract (i.e., chronic sinusitis, serous otitis and…

Considerations for Prescribing Avacopan for ANCA-Associated Vasculitis

Michele B. Kaufman, PharmD, BCGP | October 27, 2021

In early October, the FDA approved avacopan to treat anti-neutrophil cytoplasmic antibody associated vasculitis. Here are insights into the treatment’s risks and considerations.

Vasculitis Guidelines in Focus, Part 3: EGPA

Michael Putman, MD | September 14, 2021

In this third article in the series, we talk with Philip Seo, MD, MHS, about eosinophilic granulomatosis with polyangiitis (EGPA).

Vasculitis Guidelines in Focus, Part 2: ANCA-Associated Vasculitis

Michael Putman, MD | September 9, 2021

Sharon Chung, MD, MAS, discusses specific recommendations for the treatment and management of ANCA-associated vasculitis from the latest ACR Guideline.

3 New Vasculitis Guidelines Discussed

Ruth Jessen Hickman, MD | May 13, 2021

The ACR, in concert with the Vasculitis Foundation, released three new vasculitis guidelines online first in July, with a fourth slated for publication by the end of the year. These are the first ever to be produced and endorsed by the ACR and the Vasculitis Foundation. Although most of the recommendations are conditional, due to…

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