Granulomatosis with polyangiitis

[Tracie Lynn]

Granulomatosis with polyangiitis often begins with inflammation of small- and medium-sized blood vessels and tissues in the nose, sinuses, throat, lungs, or kidneys.

The cause is unknown.
The disorder usually begins with nosebleeds, nasal congestion with crusting, sinusitis, hoarseness, ear pain, fluid in the middle ear, eye redness and pain, wheezing, and coughing.
Other organs may be affected, sometimes with serious complications, such as kidney failure.
Symptoms and other findings suggest the diagnosis, but a biopsy is usually needed to confirm it.
A corticosteroid and a second drug that suppresses the immune system are needed to control inflammation and induce remission.

(See also Overview of Vasculitis.)
Granulomatosis with polyangiitis is most common among whites but can occur in all ethnic groups and at any age. Most people are affected at about age 40. Its cause is unknown. It resembles an infection, but no infecting organism has been identified. Collections of immune cells that cause inflammation (called granulomas) form nodules and ultimately destroy normal tissue. Granulomatosis with polyangiitis is often life threatening.