Dangers of Untreated Granulomatosis with Polyangiitis

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adult male patient describing his nasal problem to a physician

Granulomatosis with polyangiitis, or GPA, is a form of vasculitis—an inflammation of the blood vessels that can damage organs in your body. Granulomas are masses of immune cells that form when there is infection or inflammation. Polyangiitis means there are multiple inflamed blood vessels. It was once named Wegener's granulomatosis for a German doctor who studied the condition.

Granulomatosis with polyangiitis is a serious disease that, left untreated, can lead to permanent organ damage or even be fatal. When GPA is treated quickly, most people recover, though there is a high rate of recurrence. Here’s what to know about untreated granulomatosis with polyangiitis.

Symptoms of Granulomatosis With Polyangiitis

No one knows what causes GPA, which can occur at any age and appears in men and women equally. The inflammation limits blood flow to organs, which causes damage to them. GPA most commonly affects the nose, sinuses, windpipe, lungs and kidneys. It can also affect skin, which may leave scars, and it can weaken the cartilage in your nose, affecting the shape of the bridge. GPA can also cause hearing loss. Without treatment, kidney or lung failure can occur, which can be fatal.

The first warning signs of GPA often appear in your sinuses, throat or lungs. They can appear suddenly or over a period of months, but the condition often worsens rapidly which is why quick treatment is so important.

Typical symptoms include:

  • A runny, crusty nasal drainage that doesn’t improve
  • Nosebleeds or sinus infections
  • Coughing, sometimes with bloody phlegm
  • Inflamed ears, hearing problems

See your doctor if you have a runny nose that doesn't get better when you take over-the-counter cold medicines, especially if it's accompanied by nosebleeds and discharge, coughing up blood, or other warning signs of granulomatosis with polyangiitis. Your doctor can use blood and urine tests, x-rays, and tissue samples to diagnose GPA.

Treatment for Granulomatosis with Polyangiitis

When treated promptly with a combination of steroids and immunosuppressants, more than 90% of patients improve within a few months, and 75% of people go into remission, when there are no signs or symptoms of the disease. If organs become damaged, you may have other treatments as well. Unfortunately, relapses are common with granulomatosis with polyangiitis.

After you recover, your doctor can prescribe immunosuppressants that you can take long term. These medications can reduce the chance of a flare, but about half the people who go into remission will have a recurrence. Your doctor can treat a GPA flares with the same or similar types of medication. If your kidneys have been damaged, you may receive plasmapheresis—an exchange of plasma in your blood that can help your kidneys improve.

GPA is a serious disease but when treated quickly, the chance of permanent damage can be avoided and the outlook for recovery is good.

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Medical Reviewer: William C. Lloyd III, MD, FACS
Last Review Date: 2020 May 21
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