Granulomatosis with polyangiitis (GPA)/Wegener's granulomatosis

Overview

GPA, formerly known as Wegener’s granulomatosis, is an autoimmune vasculitis disease that causes the blood vessels of your nose, sinuses, throat, lungs, and kidney to inflame, resulting in the slowing of blood flow to specific organs. This damages organs and vessels as small areas of inflammation (granulomas) are formed.

Common Symptoms

Pus-like drainage from the nose, nose stuffiness, sinus infections, nosebleeds, coughing, shortness of breath, fever, fatigue, joint pain, numbness in bodily extremities, weight loss, blood in urine, skin sores, bruising, rashes, eye redness/burning/pain, vision problems, ear inflammation, hearing problems, and malaise.

Coexisting Diseases and Conditions

Kidney (renal) disease, polyarthralgia, arthritis, myositis, myalgia, conjunctivitis, corneal ulcerations, scleritis, episcleritis, Raynaud’s phenomenon, Goodpasture’s syndrome, gangrene, peripheral neuropathy, mononeuritis multiplex, cranial neuritis, pericarditis, endocarditis, cardiomyopathy, microscopic polyangiitis, Churg-Strauss syndrome, polyarteritis nodosa, and lymphomatosus granulomatosis.

Risk Factors and Prevalence

While GPA can affect people of any age, those most typically affected are between 40 and 65. Genetic predisposition is a commonly understood risk factor, but it is rare that more than one member of the same family will be afflicted with the disorder. Infections, environmental factors, and cigarette smoking are also believed to be linked causes.

Adult males and females contract GPA at nearly the same rate, but when diagnosed during childhood, girls have been found to be more frequently affected than boys. Some studies also have shown that males have a higher incidence of severe disease while females have more localized cases.

The disorder is underdiagnosed, so the actual numbers and groups of those affected are unknown but Caucasians have been found to be impacted most often.