Granulomatosis with Polyangiitis

Granulomatosis with Polyangiitis

What is granulomatosis with
polyangiitis?

Granulomatosis with polyangiitis (GPA), formerly called Wegener’s granulomatosis, is an
autoimmune disorder. An autoimmune disorder occurs when the body’s immune system
attacks and destroys healthy tissue. This condition causes inflammation, swelling, and
irritation in blood vessels and other tissues.

This
inflammation reduces or stops the flow of blood to organs in the body. The condition
mostly affects the respiratory system. This includes the sinuses, nose, windpipe, and
lungs. It can also affect the kidneys. But it can damage any organ in the body.

What causes granulomatosis with
polyangiitis?

GPA is not common. It can start at any
age. Doctors don’t know what causes it.

What are the symptoms of
granulomatosis with polyangiitis?

Most people with GPA first have vague
symptoms that may include:

  • Tiredness or exhaustion
  • Joint
    pain
  • Upper
    respiratory symptoms that don’t respond to treatments for allergies or colds:

    • Runny nose
    • Bloody discharge
    • Sinus pain
    • Congestion
    • Blockage of the eustachian tubes in the ears
    • Cough
    • Cough that produces blood
    • Shortness of breath
  • Eye
    infections, redness, burning, or pain
  • Weakness
  • Skin
    sores or ulcers
  • Hoarseness
  • Fever
  • Night
    sweats

How is granulomatosis with
polyangiitis diagnosed?

To diagnose GPA, your healthcare
provider may do the following:

  • Take
    your health history
  • Do a
    physical exam
  • Review
    of your symptoms
  • Order
    blood tests
  • Order
    an antineutrophil cytoplasmic antibody (ANCA) blood test
  • Order
    imaging tests, such as chest X-rays or CT scans of the lungs or sinus
  • Do a
    biopsy from an affected organ to see if the condition is present
  • Order
    urine tests

Some
of these tests can help rule out other causes of your symptoms. They may not confirm the
diagnosis. A biopsy is the only way to know for sure if it’s GPA.

How is granulomatosis with
polyangiitis treated?

Most
people with GPA can be treated. But the medicines used to treat this condition could
have side effects of their own. Be sure to talk about these side effects with your
healthcare provider.

These
are common medicines used in treatment:

  • Corticosteroids. This steroid helps reduce inflammation. Long-term steroid
    use can affect your bone health, so your healthcare provider may also watch and treat
    changes in your bone density.
  • Rituximab. This is an antibody against certain immune cells (B-cells). These
    cells help cause the autoimmune state in GPA.
  • Cyclophosphamide . This is used to treat active GPA.
    • This medicine may be given to keep symptoms in remission for 1 to 2
      years. 
    • This is an alternative to methotrexate.
    • This medicine may be given along with prednisone to treat GPA and bring about
      remission.
  • Antibiotics. Certain infections are more common among people with this
    condition. Antibiotics may be given to treat or prevent infection.

GPA
may come back even after successful treatment. Continue to follow up with your
healthcare provider, even when you are in remission.

What are the possible
complications of granulomatosis with polyangiitis?

Ongoing GPA can cause:

  • Collapse of cartilage in the nose
  • Infections of sinuses and ears
  • Hearing
    loss
  • Kidney
    damage
  • Kidney
    failure
  • Death

When should I call my healthcare
provider?

Call
your healthcare provider if you have the symptoms listed above. This is especially true
if you have tried over-the-counter treatments that seem appropriate, such as allergy
medicines, with no success.

Always call your healthcare provider if you have changes in vision. Call if you have a
cough that produces bloody mucus. These may be symptoms of granulomatosis with
polyangiitis (GPA) or another serious health problem.

Key points about granulomatosis
with polyangiitis

  • Granulomatosis with polyangiitis (GPA) is an autoimmune disorder that causes
    swelling and irritation in blood vessels and other tissues. It is uncommon.
  • Doctors
    don’t know what causes it.
  • Most
    people with GPA first report vague symptoms.
  • Biopsy
    is the only way to know for sure if it’s GPA.
  • Most
    people with GPA will find relief by taking strong medicines.
  • GGPA
    may come back even after successful treatment.
  • Ongoing
    GPA can have serious complications.
  • Continue to follow up with your healthcare provider, even when you are in
    remission.

Next steps

Tips
to help you get the most from a visit to your healthcare provider:

  • Know
    the reason for your visit and what you want to happen.
  • Before
    your visit, write down questions you want answered.
  • Bring
    someone with you to help you ask questions and remember what your provider tells
    you.
  • At the
    visit, write down the name of a new diagnosis, and any new medicines, treatments, or
    tests. Also write down any new instructions your provider gives you.
  • Know
    why a new medicine or treatment is prescribed, and how it will help you. Also know
    what the side effects are.
  • Ask if
    your condition can be treated in other ways.
  • Know
    why a test or procedure is recommended and what the results could mean.
  • Know
    what to expect if you do not take the medicine or have the test or procedure.
  • If you
    have a follow-up appointment, write down the date, time, and purpose for that
    visit.
  • Know how
    you can contact your provider if you have questions.