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Granulomatosis with polyangiitis; Other names: Wegener's granulomatosis (WG) (formerly) Micrograph showing features characteristic of granulomatosis with polyangiitis – a vasculitis and granulomas with multi-nucleated giant cells. H&E stain. Specialty: Immunology, rheumatology Causes: Autoimmune disease
In the setting of systemic vasculitis as described above, proliferative nephritis is associated with antineutrophil cytoplasmic antibodies (ANCA). [3] Because of this, an ANCA test should always follow a negative immunofluorescence result to have the highest accuracy for confirming pauci-immune vasculitis-driven proliferative nephritis.
Friedrich Wegener (7 April 1907, Varel – 9 July 1990, Lübeck, [veːɡɐnəɐ̯]) was a German pathologist who is notable for being a high-ranking Nazi physician and for his description of a rare disease originally referred to Wegener disease and now referred to as granulomatosis with polyangiitis. Although this disease was known before ...
The disease spectrum varies from failure of multiple organs to involvement of a single organ. Almost any organ could be impacted; however, polyarteritis nodosa rarely affects the lungs for unknown reasons. [10] Kawasaki disease is a type of systemic vasculitis of medium-sized vessels with an acute onset that primarily affects young children.
ANCA will less commonly form against alternative antigens that may also result in a p-ANCA pattern. These include lactoferrin, elastase, and cathepsin G. [citation needed] When the condition is a vasculitis, the target is usually MPO. [1] However, the proportion of p-ANCA sera with anti-MPO antibodies has been reported to be as low as 12%. [2]
The presence of c-ANCA is the main cause of the disease, and if it keeps attacking the granules from within neutrophils, there is a good chance the disease will return. Another small-vessel vasculitis that is very similar to granulomatosis with polyangiitis is microscopic polyangiitis.
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Eosinophilic granulomatosis with polyangiitis consists of three stages, but not all patients develop all three stages or progress from one stage to the next in the same order; [7] whereas some patients may develop severe or life-threatening complications such as gastrointestinal involvement and heart disease, some patients are only mildly affected, e.g. with skin lesions and nasal polyps. [8]