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…
The past five years have been busier than usual for the Churg-Strauss syndrome. It was renamed eosinophilic granulomatosis with polyangiitis (EGPA).1 Longitudinal cohorts totaling 484 patients—approximately as many as all previous series combined—were described.2,3 A proposal was advanced to remove and rename a subset in which vasculitis may not be present.4 And shortly after the…
Granulomatosis with polyangiitis (GPA) was first described in the British Medical Journal in 1897 by Scottish otolaryngologist Peter McBride.1 GPA is a relatively rare, systemic necrotizing vasculitis that can make diagnosis challenging. The incidence has been estimated anywhere between two and 12 cases per million.2 GPA mainly affects adults between the ages of 45 and…
For the 2024 Image Competition, the ACR sought images representing a diverse range of patients with educational or remarkable manifestations of pediatric rheumatic diseases, including autoimmune, inflammatory, infectious and malignant drivers of pediatric rheumatic disease. Here, we showcase the images selected as Best Overall. Cavitary Lung Nodules with Sinus & Skin Disease in Pediatric ANCA-Associated…
Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis is a rare autoimmune condition characterized by inflammation of small- and medium-sized vessels. ANCA-associated vasculitis can lead to multisystem organ complications, including life-threatening pulmonary hemorrhage, renal failure and death. We present a case of ANCA-associated vasculitis complicated by complement-mediated thrombotic microangiopathy (c-TMA). Of the rheumatic diseases, systemic lupus erythematosus…
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.
BALTIMORE—2022 was an exciting year in the field of vasculitis, not least of all because the ACR and EULAR released classification criteria for several forms. This development came just one year after the release of ACR/Vasculitis Foundation (VF) guidelines on the management of a number of vasculitides. With so much new information to be absorbed,…
The 19th Annual Johns Hopkins Advances in the Diagnosis and Treatment of Rheumatic Diseases Symposium session titled, Updates on Vasculitis provided an overview of the latest information on vasculitis diagnosis and treatment.
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…
Polyarteritis nodosa (PAN) is a necrotizing vasculitis, predominantly involving medium-sized arteries, that causes systemic disease, and, less commonly, cutaneous-limited disease. The population prevalence for PAN ranges from 2 to 33 per million.1-3 Estimates vary due to the increased recognition and classification of other forms of vasculitides over time and variation in the regional prevalence of…