Granulomatosis with Polyangiitis (GPA)

Jenny Ewen, BA, NREMT
Editor-In-Chief
Granulomatosis with polyangiitis (GPA), also known as Wegener’s Disease or Wegener’s Granulomatosis, is a blood vessel disorder called vasculitis (which means blood vessel inflammation). It causes inflammation and damage of the blood vessels in certain areas of the body, including the nose, sinuses, throat, lungs, and kidneys; however, it can occur anywhere in the body.

The first case of GPA was explained by Heinz Klinger (a German medical student) in 1931. Several years later it was recognized as a form of vasculitis. GPA produces an inflammatory tissue called a granuloma around the blood vessels, which can then damage normal tissue. The disease is rare, and there’s no certain cause for it. When blood vessels become inflamed, they get weak and can stretch, which can either form an aneurysm, or cause the blood vessel to rupture and bleed into the surrounding tissue. Vasculitis can also cause the blood vessel to narrow so much that the vessel becomes and closed off and blood can’t get through – then organs receiving blood and nutrients through that vessel no longer receive any, and it results in organ damage.

GPA typically occurs in middle-age, almost equally among the sexes, but can occur at any age. It’s pretty unusual for a child to develop GPA, but those as old as 70 or 80 can receive a diagnosis of GPA. There is no known cause for GPA, since it’s not a cancer, not contagious, and there’s been no evidence of it being a genetic disease. Researchers suggest the immune system plays a role in developing GPA, since the immune system is what causes blood vessel/tissue inflammation.

Signs and Symptoms

Like many other diseases, the signs and symptoms of GPA vary among patients. Symptoms can develop slowly over time or abruptly – typically with warning signs involving the respiratory tract. Some patients may only get symptoms in the lungs. Some general signs of GPA include:

  • Loss of appetite and weight loss
  • Fever
  • Fatigue or general malaise/aches and pains

More specific signs and symptoms of GPA that may develop quickly and worsen rapidly include:

  • Numbness in extremities
  • Blood in urine
  • Skin sores/bruising
  • Eye redness/burning/pain
  • Ear infections, pain, or hearing loss
  • Problems with sinuses – nosebleeds, runny nose, congestion
  • Coughing (bloody phegm may be present)
  • Shortness of breath/wheezing
  • Blood in urine
  • Kidney inflammation

Diagnosis

Diagnosis of GPA can be difficult due to the symptoms being similar to many other disorders. Early diagnosis is essential for managing the disease so it does not become fatal. Diagnosis requires a biopsy of the affected tissue. While some biopsies are relatively non-invasive, those sites don’t always give a definitive diagnosis. The best way to diagnose GPA is with a lung biopsy or kidney biopsy.

ANCA (anti-neutrophil cytoplasmic antibodies) tests can be performed with a blood test, but can occasionally result in a misdiagnosis when the test isn’t interpreted correctly. Typically an ANCA test is the first step in diagnosis, followed by a biopsy of tissue. Lung imaging can also be performed (X-ray or CT scan), even if there are no symptoms of lung disease.

Treatment

GPA is treated using medications that suppress the immune system (immunosuppressive medications). Up until the 1970s, GPA was almost always fatal, with only steroids to help prolong patients’ lives. Cyclophosphamide began to improve the prognosis of patients with GPA when used along with prednisone. GPA can go into remission with medications; however, relapses often occur – at least half of patients with GPA will suffer relapses while in remission.

Treatment also depends on the severity of GPA symptoms, and mitigating what symptoms the patient can deal with from the medications. Once in remission, doctors will reduce immunosuppressive medication dosages or change the medication to one that is less abrasive. Immunosuppressive medications are used in chemotherapy and to prevent organ transplant rejection, so there are many side effects involved with the immune system becoming suppressed. After one or two years of remission, medications may be reduced down to discontinuing them.

When someone with GPA relapses, the treatment is similar to when they were first diagnosed, with rapid medication and regular physician follow-up. Treatment is life-saving for almost everyone diagnosed with GPA. More than 80% of patients diagnosed with GPA are alive at least eight years later, and long-term survival has been seen with relatively normal lives.

Sources & More Information

Arthritis Foundation, “Wegener’s Granulomatosis (GPA)” https://www.arthritis.org/about-arthritis/types/wegeners-granulomatosis/

Cleveland Clinic, “Wegener’s Granulomatosis (GPA)” https://my.clevelandclinic.org/health/articles/wegeners-granulomatosis

Johns Hopkins Medicine – The Johns Hopkins Vasculitis Center, “Granulomatosis with Polyangiitis” https://www.hopkinsvasculitis.org/types-vasculitis/granulomatosis-with-polyangiitis/

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