The introduction of cyclophosphamide for treatment and the detection of antineutrophil cytoplasmatic antibodies (ANCA) as a seromarker for ANCA-associated vasculitis (AAV) have been the most important milestones in the history…
The outcome in patients with antineutrophil cytoplasm antibody (ANCA)-associated vasculitis has considerably improved with the use of potent immunosuppression. In the most severe cases, mortality remains an important risk despite…
Pauci-immune necrotizing glomerulonephritis is the most frequent cause of rapidly progressive glomerulonephritis and, in most cases, is associated with antineutrophil cytoplasmic antibodies (ANCA). It is either the renal manifestation of…
The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides—Wegener’s granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome—can present with various ophthalmic manifestations. In a subset of patients, these findings may be the earliest indicators of…
Churg-Strauss syndrome (CSS) is a systemic necrotizing vasculitis affecting small to medium-sized vessels, and characterized by asthma, blood hypereosinophilia, and eosinophil-rich granulomatous inflammation of the respiratory tract. In the past…
In 1923, Friedrich Wohlwill described two patients with a “microscopic form of periarteritis nodosa,” which was distinct from the classical form. This disease, now known as microscopic polyangiitis (MPA), is…
Wegener’s granulomatosis (WG) is characterized by granulomatous lesions and vasculitic disease manifestations. Granulomatous lesions are found in the upper and lower respiratory tract (eg, granulomatous sinusitis, orbital masses, and pulmonary…
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) include Wegener’s granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome. Given their rarity, protean clinical manifestations, imperfect diagnostic tests, and wide differential diagnosis, they pose a diagnostic…
Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides are systemic or more limited conditions characterized by necrotizing destruction of small and medium-sized vessels (eg, capillaries, venules, and arterioles). ANCAs are the most predominant…
Antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) comprises a group of systemic inflammatory vasculitides associated with circulating autoantibodies directed against the neutrophil granule components proteinase 3 and myeloperoxidase. ANCA interact with…
