Knowra Eosinophilic granulomatosis with polyangiitis Eosinophilic granulomatosis with polyangiitis Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic disease combining asthma, eosinophil-rich inflammation, and vasculitis of small and medium blood vessels. It can damage the lungs, skin, peripheral nerves, heart, and other organs.
Eosinophils : Eosinophils are white blood cells involved in immune defense and inflammatory responses, especially those associated with parasites and allergic disease. These cells accumulate in affected tissues and contribute to EGPA’s characteristic inflammation.
Eosinophilia : Eosinophilia is an abnormally high number of eosinophils in blood or tissues. Blood eosinophilia commonly supports the diagnosis and can track a major component of EGPA activity.
Granulomatosis with polyangiitis : Granulomatosis with polyangiitis is a systemic vasculitis that commonly affects the upper airways, lungs, and kidneys. Unlike EGPA, it is not typically defined by asthma and marked eosinophilia.
Jacob Churg : Jacob Churg was a pathologist who, with Lotte Strauss, described a distinctive eosinophilic vasculitis in 1951. The disease was historically named Churg–Strauss syndrome after his contribution.
Antineutrophil cytoplasmic antibodies : Antineutrophil cytoplasmic antibodies are autoantibodies directed against proteins in neutrophils and related cells. A subset of people with EGPA has these antibodies, which are associated with particular disease patterns.
Peripheral neuropathy : Peripheral neuropathy is damage or dysfunction of nerves outside the brain and spinal cord. EGPA-related nerve inflammation can cause painful, asymmetric weakness or sensory loss.
Microscopic polyangiitis : Microscopic polyangiitis is a small-vessel vasculitis that can affect the kidneys, lungs, skin, and nerves. It can resemble EGPA’s vasculitic phase but usually lacks its asthma-eosinophilia pattern.
Lotte Strauss : Lotte Strauss was a pathologist who, with Jacob Churg, described a distinctive eosinophilic vasculitis in 1951. Her work helped establish the clinical and pathological pattern now called EGPA.
Small-vessel vasculitis : Small-vessel vasculitis is inflammation of vessels such as capillaries, venules, and arterioles. EGPA’s vascular inflammation often targets small vessels and can impair blood supply to organs.
Myocarditis : Myocarditis is inflammation of the heart muscle, which can impair the heart’s ability to pump or conduct electrical signals. Cardiac inflammation is a serious EGPA complication and may occur without obvious symptoms.
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