Wegener’s disease, now officially called granulomatosis with polyangiitis (GPA), is a rare autoimmune condition that inflames and damages small blood vessels throughout the body. It most commonly targets the sinuses, lungs, and kidneys, and without treatment it can be life-threatening. The name was changed in 2010 after the physician it was named for, Friedrich Wegener, was found to have links to Nazism. You’ll still hear both names used, but GPA is the current medical term.
How GPA Affects the Body
GPA is driven by a malfunction in the immune system. In most patients, the body produces abnormal antibodies called ANCA (antineutrophil cytoplasmic antibodies). These antibodies latch onto a type of white blood cell called a neutrophil and essentially activate it in the wrong place at the wrong time. Once activated, these neutrophils stick to the walls of small blood vessels, release toxic enzymes and inflammatory chemicals, and damage the vessel lining from the inside out.
The result is widespread inflammation of small blood vessels, a process called vasculitis. As the vessel walls become damaged, blood flow to nearby tissues is reduced, leading to clusters of inflamed immune cells (granulomas) and areas of tissue death. This can happen in virtually any organ, but it has a strong preference for the upper airways, lungs, and kidneys.
Common Symptoms by Organ
GPA often starts with symptoms that look like a stubborn sinus infection or cold that simply won’t go away. About 90% of patients experience upper respiratory problems: chronic nasal congestion, bloody nasal discharge, crusting inside the nose, and recurring sinus infections. Over time, the cartilage in the nose or ears can be damaged, sometimes causing a visible change in the shape of the nose (called a saddle nose deformity). Hearing loss, either from fluid buildup or nerve damage, is another recognized feature.
Lung involvement shows up in roughly half of patients at the time of diagnosis. This can range from a persistent cough and shortness of breath to coughing up blood. Chest imaging often reveals nodules, masses, or cavities in the lungs that can mimic the appearance of cancer or tuberculosis.
Kidney disease is the complication that most affects long-term outcomes. Only 10% to 20% of patients have kidney involvement when they’re first diagnosed, but within two years, about 80% develop a form of kidney inflammation called glomerulonephritis. Because the kidneys can lose significant function before symptoms become obvious, this is one of the most important things doctors monitor.
Other possible symptoms include joint pain, skin rashes or ulcers, red or painful eyes, and numbness or tingling from nerve damage. The disease can also cause fatigue, fevers, and unintentional weight loss.
Who Gets GPA
GPA is uncommon. In working-age adults in the United States, the incidence is roughly 13 cases per million people per year. In children, it’s far rarer, at about 2 cases per million per year. The median age at diagnosis is 53 in adults and 15 in the small number of children affected. It occurs slightly more often in men and is most prevalent in people of Northern European descent, though it can affect anyone.
How GPA Is Diagnosed
Diagnosis typically involves a combination of blood tests, imaging, and tissue biopsy. The most important blood test looks for ANCA antibodies, specifically a subtype called PR3-ANCA. These antibodies are found in about three-quarters of GPA patients and are highly specific to the disease (over 90% specificity), meaning a positive result strongly points toward GPA rather than something else. A negative result doesn’t rule it out, though, particularly in early or limited forms of the disease.
Imaging of the sinuses and lungs helps identify characteristic patterns of inflammation, nodules, or cavities. A biopsy, usually taken from the nose, sinuses, lungs, or kidneys, looks for three hallmark features: blood vessel inflammation, granulomas, and areas of tissue death. In practice, it’s uncommon for a single biopsy sample to show all three features clearly, so doctors piece the diagnosis together from the full clinical picture.
The 2022 classification criteria developed by the American College of Rheumatology assign weighted points to different findings. A positive PR3-ANCA test carries the highest weight (+5 points), followed by nasal symptoms like bloody discharge or crusting (+3), and lung nodules or cartilage damage (+2 each). A combined score of 5 or more points, after ruling out other causes of vasculitis, supports a GPA classification.
Treatment and What to Expect
GPA is treated in two phases: first bringing the disease under control (remission induction), then keeping it quiet long-term (maintenance therapy). For serious or organ-threatening disease, remission induction combines high-dose steroids with a powerful immune-suppressing medication. The two most common options work differently: one targets a specific type of immune cell (B cells) responsible for producing the harmful antibodies, while the other broadly suppresses the immune system’s ability to attack. Both approaches achieve remission in a large proportion of patients, typically assessed around six months into treatment.
Once remission is achieved, patients transition to a lower-intensity maintenance regimen that they may stay on for years. The goal is to prevent relapses, which are common in GPA. Levels of PR3-ANCA antibodies in the blood tend to correlate with disease activity, so regular blood work helps doctors spot flare-ups early.
Long-Term Outlook
Before modern immunosuppressive treatment existed, GPA was almost always fatal within a year or two. Today, the five-year survival rate is approximately 80%, and study data show survival rates of about 84% at five years and 77% at nine years. These numbers represent a dramatic improvement, though they also reflect that GPA remains a serious chronic condition.
Kidney involvement is the single biggest factor in long-term prognosis. The 10-year survival rate is roughly 60% to 70% for patients without kidney disease but drops to about 40% when the kidneys are significantly affected. This is why early detection and aggressive treatment of kidney inflammation matter so much.
Beyond the disease itself, long-term immune suppression carries its own risks, including increased susceptibility to infections and, over many years, a modestly higher risk of certain cancers. Most people with GPA need ongoing monitoring with regular blood tests and imaging for the rest of their lives, even during periods of remission. Relapses can occur years after the initial diagnosis, so staying connected with a specialist who manages vasculitis is an important part of living with this condition.