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.
Summaries of selected research abstracts on the assessment & management of systemic vasculitis from ACR Convergence 2024.
Results of the international SELECT-GCA study suggest that upadacitinib may be an effective new oral treatment for giant cell arteritis.
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…
Summaries of selected research abstracts on ANCA-associated vasculitis & IgA vasculitis from the International Vasculitis Workshop.
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…
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)…
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…
SAN DIEGO—Vasculitis expert and former editor of The Rheumatologist, Dr. Philip Seo gives us his picks for the 10 most important abstracts in ANCA-associated vasculitis to come out of ACR Convergence 2023.
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…