Granulomatosis with polyangiitis (GPA, Wegener’s granulomatosis)

Granulomatosis with polyangiitis (GPA), formerly called Wegener’s granulomatosis, is a rare form of ANCA-associated vasculitis in which the immune system attacks small- and medium-sized blood vessels. GPA commonly affects the nose, sinuses, ears, throat, lungs, and kidneys, leading to inflammation that can reduce blood flow and damage tissues and organs. The disease is characterized by the formation of granulomas, which are small areas of inflammatory tissue. Many people with GPA have anti-neutrophil cytoplasmic antibodies (ANCA), particularly PR3-ANCA.

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.

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.

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.

• Familial Association of Granulomatosis With Polyangiitis: A Case-Based Review of Literature (2023)
• Eosinophilic granulomatosis with polyangiitis: A review (2023)
• Therapeutic Advances in Eosinophilic Granulomatosis with Polyangiitis (2023)
• Updates of ocular involvement in granulomatosis with polyangiitis (2023)