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…
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,…
Granulomatosis with polyangiitis (GPA) is a primary, necrotizing granulomatous vasculitis, involving small- to medium-sized arteries, that causes systemic disease. Almost any organ can be affected, but the most affected systems are the upper airways, lungs, kidneys, eyes and peripheral nerves. Migratory polyarthritis is reported in approximately 25% of patients with anti-neutrophil cytoplasmic antibody (ANCA) associated…
Granulomatosis with polyangiitis (GPA) is a type of anti-neutrophil cytoplasmic antibody (ANCA) associated small vessel vasculitis that typically affects the kidneys, lungs and sinuses.1 Due to an overlap in signs and symptoms, GPA may initially be difficult to distinguish from IgG4-related disease, another condition that can affect multiple tissues and has variable presentations. Further complicating…
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…
In September 2019, a previously healthy, 9-year-old white girl presented to the emergency department following two months of sinusitis and unexplained fever responsive to ibuprofen. She presented with anorexia; a 9 lb. weight loss; intermittent, nonbilious, nonbloody emesis; and occasional epistaxis with digital manipulation of the nose. Six weeks prior to admission, she had presented to…
A 67–year-old white woman with primary Raynaud’s phenomenon presented following a week of progressively worsening shortness of breath, dry cough and generalized malaise. An avid tennis player, she first noticed dyspnea while playing, but a few days later grew short of breath even at rest. She went to an urgent care center, where a computed…
The FDA has approved an update for rituximab’s label, which will include safety and efficacy information for treating ANCA-associated vasculitis…
A recent study in Arthritis & Rheumatology highlights new information about the epidemiology and disease course of the vasculitic disease granulomatosis with polyangiitis (GPA, formerly known as Wegener’s disease).1 GPA is a rare disease that’s generally specific to the lungs, kidneys and the upper airways. The study provides key new data about the incidence and…
Based on the classification system developed by the Chapel Hill Consensus Conference, anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis is defined as a necrotizing vasculitis involving small vessels that is associated with myeloperoxidase (MPO) ANCA or proteinase 3 (PR3) ANCA and displays minimal immune deposits. The mechanism behind the pathogenesis of ANCA-associated vasculitis is not fully…