Granulomatosis with polyangiitis (GPA), formerly called Wegener’s granulomatosis, is a rare blood vessel disease. It’s a type of vasculitis, or inflammation of the blood vessels, specifically small- and medium-sized blood vessels causing an inability of blood to flow properly and deliver oxygen to cells around the body. Granuloma, or cellular inflammation, occurs, causing damage in the sinuses, lungs, and kidneys, but may also affect eyes, ears, skin, nerves, joints and other organs. GPA is a rare condition, affecting only 3 out of every 100,000 people, typically aged 40 to 65 and equally affecting women and men.

What Are the Signs/Symptoms?

GPA symptoms may develop over days or months. Early signs are nasal congestion, frequent nosebleeds, shortness of breath and coughing up bloody phlegm. Other possible signs are joint pain, decreased hearing, skin rashes, eye redness or vision changes, fatigue, fever, loss of appetite and weight, night sweats, and numbness or loss of movement in fingers, toes or limbs. A rheumatologist can diagnose GPA with a physical examination, lab tests, imaging scans and biopsy. A positive blood test for anti-neutrophil cytoplasmic antibodies (ANCA) suggests but doesn’t confirm GPA. Up to 20% of people with a diagnosis of GPA test negative for ANCA. If GPA is suspected, further evaluation will likely occur. Tissue biopsy confirms GPA diagnosis.

What Are Common Treatments?

GPA can lead to kidney or lung failure without prompt treatment. Treatment choice depends on the organs involved, disease severity and a person’s overall health and may include steroid, disease modifying rheumatic drugs (DMARDs), chemotherapy, or biologic based treatment as determined by their rheumatology healthcare provider. The rheumatologist and patient can talk about the best treatment options, as these medications may have serious side effects.

Living with GPA

People with GPA need regular check-ups, as disease relapses are common. It can also have serious complications that are even life-threatening if not treated promptly. Patients need regular laboratory tests, imaging scans and clinical visits with a rheumatology provider. Relapses of GPA may involve different symptoms than earlier episodes, so patients should report new symptoms to their rheumatology provider right away. Regular rheumatology visits and testing should spot any problems early so that prompt, effective treatment can be started.