Granulomatosis with polyangiitis (GPA) is an uncommon autoimmune disorder that makes blood vessels become inflamed.
Most often diagnosed in middle-aged adults, granulomatosis with polyangiitis is rarely seen in children. Its cause is unknown.
Some people with GPA may develop small nodules, especially around blood vessels, called granulomas. Blood vessel inflammation affects blood flow, which can damage organs.
Symptoms usually affect the respiratory system first (sinuses, throat, and lungs). The condition progresses quickly, though, and can be life-threatening if it affects the kidneys. Many people with GPA also develop eye problems.
People with GPA may have:
Coughing up blood or blood in the urine also can be signs of GPA.
Doctors treat granulomatosis with polyangiitis with a variety of medicines, such as steroids and others that suppress the immune system. The medicines are effective, but the disease can come back, and treatment can have side effects. So patients with GPA will be closely watched by their doctor.
All A to Z dictionary entries are regularly reviewed by KidsHealth medical experts.
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