Wegener's Syndrome - Granulomatosis with Polyangiitis (pathophysiology, symptoms, treatment)

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Case description

Wegener's Syndrome is the former name, this condition should be known as Granulomatosis with Polyangiitis "Granulomatosis with polyangiitis also known as Wegener's syndrome is the most common AAV and 95% of patients are ANCA positive mostly against PR3+. It is a small vessel vasculitis characterized by inflammation of the small blood vessels with infiltration of immune cells forming granuloma. The granulomas as mentioned are formed by T-cells and other immune cells. Granulomatous inflammation occurs in the blood vessels of the upper respiratory tract, lower respiratory tracts and kidneys. This is Wegener's triad – Upper RT lower RT and renal tract involvement. Wegener's syndrome is the most prevalent of the AAVs with a mean age of onset of 40, but really can occur at any age. It occurs in females and males equally. Histopathological findings include pauci immune necrotising vasculitis of small arteries and veins together with granuloma formation.


Video by: Armando Hasudungan 

tags: Wegener's Syndrome vasculitis granulomatosis with polyangiitis


Natalia Dułak
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Natalia Dułak

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