Health A-Z

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What Is It?

Granulomatosis with polyangiitis (Wegener's) is a relatively rare disease marked by inflammation of the blood vessels. The condition is potentially life-threatening.

In Granulomatosis with polyangiitis (Wegener's), inflammation damages the walls of small- and medium-sized arteries and veins. This damage interferes with normal blood supply to nearby tissues. As a result, tissues in many parts of the body may be injured and destroyed.

Tissues affected by Granulomatosis with polyangiitis (Wegener's) contain islands of inflamed cells. These are called granulomas.

Three areas in the body are affected most commonly:

  • Upper airways (including the sinuses, trachea and nose). In the upper airways, tissue destruction causes chronic (long-lasting) nasal and sinus problems. Many people with Granulomatosis with polyangiitis (Wegener's) first see their doctors because of sinusitis, persistent runny nose or frequent bloody nose.

  • Lungs. In most people with Granulomatosis with polyangiitis (Wegener's), inflammation targets the lungs as well. This causes a respiratory illness. Symptoms include coughing, shortness of breath, wheezing or coughing up blood.

  • Kidneys. Kidney damage affects most people with Granulomatosis with polyangiitis (Wegener's). But in many cases, this damage is mild and does not cause any symptoms. In some people, however, the kidney damage is more severe. It may cause kidney failure.

In some cases, Granulomatosis with polyangiitis (Wegener's) involves other organs. This may include the eyes, ears, skin, joints, heart and nerves.

Granulomatosis with polyangiitis (Wegener's) is believed to be an autoimmune disorder. This means the body's immune defenses mistakenly attack the body's own cells and tissues. In this case, the misdirected immune attack is aimed at the blood vessels. This triggers inflammation and damage.

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From Health A-Z, Harvard Health Publications. Copyright 2007 by the President and Fellows of Harvard College. All rights reserved. Written permission is required to reproduce, in any manner, in whole or in part, the material contained herein. To make a reprint request, contact Harvard Health Publications. Used with permission of StayWell.

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