Granulomatosis with polyangiitis (GPA, formerly Wegener's)

Granulomatosis with polyangiitis (GPA) is inflammation of small and medium blood vessels with clusters of inflammatory tissue called granulomas, in which the immune system appears to attack the vessels and surrounding tissue.

It is the most common of the ANCA-associated vasculitides, a group of conditions linked to antineutrophil cytoplasmic antibodies, and most people with GPA test positive for the PR3-ANCA antibody. It commonly affects the sinuses, nose, lungs, and kidneys, and can involve other organs as the inflammation spreads. GPA is considered a rare disease, and estimated incidence varies by country, from roughly 10 to 12 cases per million person-years in the United States and United Kingdom to lower rates elsewhere. A large United States claims-database analysis found an incidence of about 12.8 cases per million person-years in working-age adults and 1.8 per million in children, and it can occur at any age, with some studies describing a slight female predominance.

Common signs

Persistent runny or crusty nose, sinus pain, nosebleeds, ear problems, cough or shortness of breath that can include coughing up blood, kidney involvement, red or painful eyes, joint aches, fatigue, and fever.

This is a plain-language summary for orientation, not a diagnosis. Mechanisms are described as current understanding, not settled fact. Work with your own clinician for your care.