(Granulomatosis with Polyangiitis)
Wegener's Granulomatosis Overview
Wegener's granulomatosis (WG) also known as granulomatosis with polyangiitis (GPA) is a rare disease in which blood vessels become inflamed (a condition called vasculitis) and localized, nodular collections of abnormal inflammatory cells, known as granulomas, are found in affected tissues. Granulomatosis is the term that refers to the presence of granulomas. The inflammation can affect various parts of the body, such as the respiratory tract (nose, sinuses, windpipe, and lungs) and the kidneys. When blood vessels become inflamed, blood flow to the organs is disrupted, which can damage the organs. The condition is named after Friedrich Wegener, who identified the illness in the 1930s.
Wegener's granulomatosis is rare, and its exact frequency is difficult to determine.
The disease can affect anyone at any age. However, it affects white people in
their 30s and 40s most often.
With proper diagnosis and treatment, the disease can be slowed or stopped.
Wegener's Granulomatosis Causes
No one knows the cause of Wegener's granulomatosis. It is thought to be an autoimmune disorder in which the body's defense system attacks itself and destroys normal body tissue. Normally, the body's immune system acts as a defense against outside invaders. With an autoimmune disorder, something triggers an immune response causing the body to react against its own tissue. The result is that tissue can be damaged. With Wegener's granulomatosis, the organ systems that are attacked include the respiratory tract and kidneys.
Medically Reviewed by a Doctor on 9/15/2014
Sat Sharma, MD, FRCPC, FCCP
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