Wegeners Syndrome - Granulomatosis with Polyangiitis (Pathophysiology, Symptoms, Treatment)

Granulomatosis with polyangiitis also known as Wegeners syndrome is the most common AAV and 95% of patients are ANCA positive mostly against PR3+. It is a small vessel vasculitis characterised 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 the Wegener's triad – Upper RT lower RT and renal tract involvement. Wegeners 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.