Granulomatosis with Polyangiitis (GPA)

Overview

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a rare autoimmune disease that causes inflammation of blood vessels, a process called vasculitis. The inflammation damages small and medium-sized vessels and forms clusters of inflammatory tissue called granulomas, which can injure the organs they supply.

GPA most commonly affects the upper airways (nose and sinuses), the lungs, and the kidneys, but it can involve the eyes, ears, skin, joints, and nerves. It belongs to a group of disorders called ANCA-associated vasculitis, named for an antibody often found in the blood. Although GPA can be serious, modern treatment has greatly improved outcomes, allowing many people to reach lasting remission.

The course of GPA varies from person to person. Some have mild disease limited mainly to the nose and sinuses, while others develop more widespread, severe illness that can rapidly affect the kidneys and lungs. Because the early symptoms often resemble common infections, the diagnosis can be delayed, which is one reason persistent or unusual sinus, lung, or kidney symptoms deserve careful evaluation. With ongoing care, the disease is usually controllable even though it can relapse.

Symptoms

Symptoms vary widely depending on which organs are involved and often begin in the nose and sinuses. They may include:

• Persistent runny or stuffy nose, crusting, nosebleeds, or sinus pain
• Cough, shortness of breath, or coughing up blood
• Blood in the urine or signs of kidney problems
• Red, painful eyes, vision changes, or ear problems and hearing loss
• Skin rashes or sores, joint pain, and numbness or tingling
• General symptoms such as fever, fatigue, and unintended weight loss

Symptoms can develop gradually or come on quickly, and kidney or lung involvement can become serious if untreated.

Causes

The exact cause of GPA is not known. It is an autoimmune disease, meaning the immune system mistakenly attacks the body's own blood vessels. Several factors are thought to contribute:

• Immune dysregulation: Abnormal antibodies called ANCA appear to drive inflammation of the vessel walls.
• Genetic susceptibility: Certain genetic traits may make a person more prone to the disease.
• Environmental triggers: Infections and other exposures may set off the abnormal immune response in susceptible people.

GPA is not contagious and is not caused by anything a person did. It can occur at any age but is most common in middle and older adulthood.

Risk Factors

• Middle to older age, though any age can be affected
• A personal or family history of autoimmune disease
• Certain genetic backgrounds

GPA affects people of all backgrounds, and in most cases no clear risk factor is identified.

Diagnosis

Diagnosing GPA can be challenging because symptoms mimic many other conditions. Evaluation usually combines:

• Blood tests: Including ANCA antibody testing and markers of inflammation and kidney function.
• Urine tests: To detect blood or protein that signals kidney involvement.
• Imaging: Chest X-ray or CT scans of the sinuses and lungs.
• Biopsy: A tissue sample from an affected area (such as the nose, lung, or kidney) to confirm vasculitis and granulomas.

Treatment

Treatment has two phases: bringing the disease under control (remission induction) and keeping it controlled (maintenance). Approaches include:

• Corticosteroids: To rapidly reduce inflammation, usually combined with other medicines.
• Immune-suppressing drugs: Medicines that calm the overactive immune system to induce and maintain remission.
• Targeted biologic therapy: Used to induce remission and reduce relapses in many patients.
• Supportive care: Treating infections, protecting the kidneys, and addressing complications; in severe kidney disease, dialysis or plasma exchange may be needed.

Because relapses can occur, long-term follow-up with a specialist is essential. People with GPA are often cared for by a team that may include specialists in kidney disease, lung disease, ear-nose-and-throat conditions, and immune disorders, depending on which organs are affected. Regular monitoring of blood and urine helps detect flares early so treatment can be adjusted before serious damage occurs.

When to See a Doctor

See a doctor for persistent sinus symptoms, nosebleeds, cough, or unexplained fatigue and weight loss, especially in combination. Seek emergency care immediately if you have:

• Coughing up blood or severe shortness of breath
• Visible blood in the urine or a sudden drop in urine output
• Severe, sudden vision changes
• Chest pain or signs of serious illness such as confusion or fainting

Related Topics

Frequently Asked Questions

References

1. National Institute of Allergy and Infectious Diseases (NIAID).
2. Mayo Clinic. Granulomatosis with polyangiitis — Symptoms and causes.
3. MedlinePlus, U.S. National Library of Medicine. Granulomatosis with polyangiitis.
4. Vasculitis Foundation. Granulomatosis with polyangiitis (GPA).