Granulomatosis With Polyangiitis
What is Granulomatosis with Polyangiitis?
Granulomatosis with Polyangiitis (GPA), also known as Wegener’s Granulomatosis or Wegener’s, is a rare, systemic vasculitis that can affect many organs and tissues. It is a disease in which a person’s immune system attacks the small blood vessels, leading to inflammation and possible tissue damage. If left untreated GPA can cause severe organ damage and can even be fatal.
What are the Causes?
The cause of GPA is unknown. It is thought to be an autoimmune disorder, meaning that the person’s own immune system attacks healthy body tissue. It is also thought to involve genetic factors, as it tends to run in families. However, the exact reasons why some people get GPA are still not entirely known.
What are the Symptoms?
Symptoms of GPA can vary from person to person, but the most common include:
- Fatigue
- Weight loss
- Fever
- Cough, hoarseness, or shortness of breath
- Chest pain
- Joint pain
- Swollen legs or ankles
- Peripheral neuropathy
- Eye inflammation or vision problems
- Skin rashes, lesions, or ulcers
How is GPA Diagnosed?
GPA is typically diagnosed through a combination of medical history, physical examination, laboratory test results and imaging studies. A chest X-ray, CT scan, or MRI may be ordered to monitor possible organ damage. A biopsy may also be performed to confirm a diagnosis of GPA.
How is GPA Treated?
GPA is typically treated with a combination of medications and lifestyle changes. Medications include corticosteroids, such as prednisone, and immune-suppressing medications, such as methotrexate or cyclophosphamide. Patients may also be advised to avoid activities that could cause any additional damage to their organs or tissues. In severe cases, surgery may be necessary to limit the spread of the disease or to treat any organ damage.
What is the Outlook?
The outlook for people with GPA is generally good, however long-term monitoring is important to ensure that any organ damage is monitored and treated. With proper medical treatment, the risk of death from GPA is low.