Granulomatosis with Polyangiitis Home A-Z Health Information Health Library A-Z Granulomatosis With Polyangiitis Overview Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a rare disorder characterised by inflammation of the blood vessels, which can restrict blood flow to various organs. It primarily affects the respiratory tract (upper as well as lower) and kidneys; however, it can affect other organs as well. Causes and Risk Factors The exact cause of GPA is unknown, but it is considered as an autoimmune disorder, where the body's immune system mistakenly attacks its own blood vessels. Genetic and environmental factors may contribute to its development. Symptoms Respiratory: Persistent nasal congestion, sinusitis, nosebleeds, cough, and shortness of breath.Renal: Blood in the urine, decreased kidney function, and in severe cases, kidney failure.General: Fatigue, fever, weight loss, and joint pain. Diagnosis Diagnosing GPA involves a combination of clinical evaluation, laboratory tests (including blood tests for specific antibodies like ANCA), imaging-based techniques (e.g., chest X-rays or CT scans), and sometimes biopsy of the affected tissues. Treatment GPA is typically treated with immunosuppressive medications to reduce inflammation and suppress the immune system. Commonly used drugs include corticosteroids and other immunosuppressants like cyclophosphamide, methotrexate, or rituximab. Early diagnosis and treatment are crucial to prevent organ damage.Prognosis: With prompt and appropriate treatment, the prognosis for GPA has improved significantly. However, the condition can be life-threatening if not treated early, and long-term follow-up is necessary as relapses can occur.