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Apart from GPA, this category includes eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis. [1] Although GPA affects small- and medium-size vessels, [20] it is formally classified as one of the small vessel vasculitides in the Chapel Hill system. [2]
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]
The negative immunofluorescence pattern, however, is called "pauci-immune" and is often associated with systemic vasculitides (plural of vasculitis) including: microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (EGPA), and granulomatosis with polyangiitis (GPA). [1] [2]
Granulomatosis with Polyangiitis is a relatively rare disorder, occurring in 1/25,000 persons. [9] After revelations about his Nazi Party past became common knowledge, the disease has been referred to as Granulomatosis with Polyangiitis (GPA). The American College of Chest Physicians (ACCP) awarded Wegener a “master clinician” prize in 1989 ...
Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis (WG), is a rare immune-mediated systemic disease with an unclear etiology. It manifests pathologically as an inflammatory response pattern in the kidneys, upper and lower respiratory tracts, and granulomatous inflammation, which includes necrosis. [13 ...
Eosinophilic granulomatosis with polyangiitis (EGPA) Small to medium vessels in respiratory tract, skin, heart, kidneys, nerves ANCA Confirmed [36] Granulomatosis with polyangiitis (GPA) Small to medium vessels in respiratory tract, kidneys c-ANCA/PR3-ANCA Confirmed [36] IgA vasculitis (IgAV)
The signs and symptoms of microscopic polyangiitis may resemble those of granulomatosis with polyangiitis (GPA) (another form of small-vessel vasculitis) but typically lacks the significant upper respiratory tract involvement (e.g., sinusitis) frequently seen in people affected by GPA. [citation needed]
Type 3 rapidly progressive glomerulonephritis, also called pauciimmune type, is associated with causes of vascular inflammation including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis. No immune deposits can be seen on staining, however blood tests may be positive for the ANCA antibody. [4]: 558–559