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An official publication of the ACR and the ARP serving rheumatologists and rheumatology professionals

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.

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Articles

Case Report: Pulmonary Artery Stenosis Secondary to Granulomatosis with Polyangiitis

Sambhawana Bhandari, MD, & Colin Diffie, MD  |  

This report presents a rare case of bilateral pulmonary artery stenosis secondary to granulomatosis with polyangiitis, underscoring the importance of recognizing large-vessel involvement as a potential manifestation of AAV; it also includes a narrative review analyzing eight previously reported cases.

Key Research in Vasculitis Encapsulated

Philip Seo, MD, MHS  |  

Summaries of selected research abstracts on the assessment & management of systemic vasculitis from ACR Convergence 2024.

Case Report: Giant Cell Arteritis Presenting with Scalp Necrosis

Jude Al Qaqaa, MD, Vladimir Falb, DO, & Kaitlyn Buzard, DO  |  

Giant cell arteritis (GCA), is an inflammatory vasculitis that involves large- and mediumsized arteries, most commonly the cranial branches of the carotid artery.1 It is seen in individuals 50 years and older.1 The most typical manifestation is new-onset headache with scalp tenderness and jaw claudication. 2 The most feared complication associated with GCA is permanent…

Case Report: An Uncommon Presenting Complaint of GPA

Adina Greene, BA, Sabrina Dahak, MD, Wesley A. Robertson, MD, Denege Ward-Wright, MD, & Kristen Young, DO, MEd  |  

Granulomatosis with polyangiitis (GPA) is a small vessel vasculitis associated with anti-neutrophil cytoplasmic antibodies (C-ANCA), specifically proteinase 3 (PR3) ANCA. GPA classically presents with multi-organ involvement. The first description of GPA defined a triad of features: 1) systemic necrotizing angiitis, 2) necrotizing inflammation of the respiratory tract and 3) necrotizing glomerulonephritis.1 Few cases of digital…

Trimethoprim Sulfamethoxazole Prophylaxis & Infections in Patients with GPA

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SAN DIEGO—In their study, Effect of Trimethoprim Sulfamethoxazole Prophylaxis on Infections During Treatment of Granulomatosis with Polyangiitis with Rituximab: A Population-Based Study, Mendel et al. assessed the relationship between trimethoprim sulfamethoxazole (TMP-SMX) and infection in patients undergoing treatment for granulomatosis with polyangiitis (GPA). Background/Purpose Infections during treatment of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV)…

Reduced-Dose Glucocorticoids for GPA & Microscopic Polyangiitis

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SAN DIEGO—In a study titled Real-Life Use of the PEXIVAS Reduced-dose Glucocorticoid Regimen in Granulomatosis with Polyangiitis and Microscopic Polyangiitis, Nagle et al. evaluated the efficacy and safety of the reduced-dose glucocorticoid regimen in a real-world setting. Background/Purpose Glucocorticoids (GCs) in combination with rituximab (RTX) or cyclophosphamide are the cornerstone of treatment for patients with…

RheumMadness 2023: The Results Are In

David L. Leverenz, MD, MEd  |  

RheumMadness is an online tournament in which a bracket of teams, representing key learning concepts in rheumatology, compete against each other in a series of head-to-head matchups, much like basketball teams in the NCAA’s March Madness. The 2023 tournament theme was The All Star Season. Each team represented one all star article competing to be…