Granulomatosis with Polyangiitis (GPA)

What is Granulomatosis with Polyangiitis?

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a rare form of vasculitis. In this disorder, small-sized blood vessels in the nose, sinuses, ears, lungs and kidneys become inflamed and damaged. Other areas may also be affected in some cases. It can also produce a type of inflammatory tissue known as a granuloma that's found around the blood vessels. Granulomas can destroy normal tissue.

Granulomatosis with polyangiitis is most common in middle-aged adults, with an average age of onset between 40 and 65 years. It is rare in children, but has been seen in infants as young as 3 months old. Whites are more likely than blacks to develop Granulomatosis with polyangiitis.

Granulomatosis with Polyangiitis Causes

The exact cause is unknown, but it is an autoimmune disorder. An autoimmune disorder occurs when the body’s immune system attacks and destroys healthy body tissue by mistake.

Granulomatosis with Polyangiitis Symptoms

Sinus and lung symptoms are most common in GPA. Other early symptoms include fever, night sweats, fatigue and a general ill feeling (malaise). For some people, the disease is limited to the respiratory system and doesn't involve the kidneys. Kidney involvement usually doesn't cause symptoms early in the course of the disease. It may be detected only by blood and urine tests. Over time, kidney failure and anemia often occur.

Signs and symptoms may include:

  • Sinus pain and inflammation
  • Runny nose, with pus-filled discharge
  • Nosebleeds
  • Fever
  • General ill feeling (malaise)
  • Unintended weight loss
  • Ear infections
  • Cough
  • Chest pain
  • Coughing up blood
  • Shortness of breath
  • Joint aches and swelling
  • Blood in urine
  • Skin sores
  • Eye redness, burning or pain

Granulomatosis with Polyangiitis Diagnosis

A doctor makes a suspected diagnosis based on a combination of symptoms, physical examination results, laboratory tests and X-rays. A biopsy will usually be used to confirm it.

The following tests may be performed:

  • ANCA. A blood test looks for proteins called antineutrophil cytoplasmic antibodies (ANCA), which may be present in people with this condition.
  • Urinalysis. Signs of kidney disease can be detected in the urine.
  • Biopsy. A small sample of affected tissue will be taken (from the skin, nasal membranes, sinuses, lungs, kidneys or other sites) and looked at under a microscope to confirm the diagnosis.

Other tests that may be done based on symptoms are:

  • Bronchoscopy with biopsy
  • Kidney biopsy
  • Nasal mucosal biopsy
  • Open lung biopsy
  • Skin biopsy
  • Upper airway biopsy
  • Sinus CT scan
  • Chest CT scan
  • Chest x-ray

Granulomatosis with Polyangiitis Treatment

Rapid treatment is essential in GPA because the disease can be deadly. With treatment, however, the outlook for most patients is good. Treatment includes corticosteroids to quickly reduce inflammation, as well as other medicines to slow the immune response. Medicines to treat the autoimmune response include azathioprine, cyclophosphamide and methotrexate. Other medicines sometimes used for the condition include bisphosphonate to prevent bone loss caused by prednisone, folic acid to combat side-effects of methotrexate and trimethoprim/sulfamethoxazole to prevent lung infections.

Granulomatosis with Polyangiitis Self Care

Following the treatment program is essential in GPA because untreated – or under treated – disease can lead to severe complications and death. The disease may return within 2 years of stopping treatment in about half of all patients.