Wegener's granulomatosis, also called granulomatosis with polyangiitis (GPA), is an uncommon disorder that causes inflammation of the blood vessels. This inflammation restricts blood flow to various organs.
Wegener's granulomatosis, often affects kidneys, lungs and the upper respiratory tract. The restricted blood flow to these organs can damage them. Wegener's can affect other organs, but this isn't as common, and generally isn't as serious.
Wegener's granulomatosis also produces a type of inflammatory tissue known as a granuloma that's found around the blood vessels. Granulomas can destroy normal tissue.
Early diagnosis and treatment of Wegener's granulomatosis may lead to a full recovery. Without treatment, Wegener's granulomatosis can be fatal, most commonly from kidney failure.
Some signs and symptoms may include, constantly runny nose, with pus-filled discharge, nosebleeds, sinus pain and inflammation, coughing up blood, joint aches and swelling, skin sores or fever.
For some people, the disease is limited to the lungs and doesn't involve the kidneys. Kidney involvement usually doesn't cause symptoms early in the course of the disease, and may be detected only by blood and urine tests. However, in time kidney failure and anemia often occur. As awareness of Wegener’s grows, more patients are diagnosed in the early stages of the disease when effective treatment can result in early remission and prevent organ failure.
There is no cure for GPA/Wegener’s, but early diagnosis and proper treatment is effective in reducing the symptoms of the disease and improving the quality of life and life expectancy of patients who suffer from it. The disease can be brought into remission with complete absence of all signs of disease.