Knowra Granulomatosis with polyangiitis Granulomatosis with polyangiitis Granulomatosis with polyangiitis is an autoimmune vasculitis that inflames small and medium blood vessels, often affecting the respiratory tract and kidneys. It is commonly associated with antineutrophil cytoplasmic antibodies and granulomatous inflammation.
ANCA-associated vasculitis : A group of small-vessel inflammatory diseases associated with antineutrophil cytoplasmic antibodies. Granulomatosis with polyangiitis is one of the principal diseases in this group.
Kidney biopsy : A procedure that removes a small sample of kidney tissue for microscopic examination. It can confirm pauci-immune glomerulonephritis and assess the severity of renal involvement.
Microscopic polyangiitis : A necrotizing small-vessel vasculitis commonly associated with ANCA and lacking granulomatous inflammation. It can resemble this disease in the kidneys and lungs but typically lacks respiratory granulomas.
Diffuse alveolar hemorrhage : Bleeding into the lung’s alveolar spaces, often causing breathlessness, low oxygen, and anemia. Pulmonary capillary injury can produce this life-threatening complication.
Friedrich Wegener : A German physician who described the disease later named Wegener’s granulomatosis. His early clinical and pathological descriptions gave the disease its former eponym.
Proteinase 3 : A neutrophil enzyme that is a major antigen targeted by autoantibodies in granulomatosis with polyangiitis. PR3-ANCA is strongly associated with this disease and helps characterize its immune response.
Chest computed tomography : An imaging method that uses X-rays and computer processing to create detailed cross-sectional views of the chest. It can reveal nodules, cavities, or infiltrates caused by pulmonary disease.
Eosinophilic granulomatosis with polyangiitis : A small- to medium-vessel vasculitis associated with asthma, eosinophilia, and granulomatous inflammation. Asthma and marked eosinophilia distinguish this related granulomatous vasculitis.
Subglottic stenosis : Narrowing of the airway below the vocal cords, often caused by scarring or inflammation. Inflammation may produce this airway complication, sometimes even when other disease signs are controlled.
Chapel Hill Consensus Conference : An international process that develops nomenclature and definitions for systemic vasculitides. Its 2012 nomenclature adopted the current name, granulomatosis with polyangiitis.
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