What is Granulomatosis with Polyangiitis?
Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is an uncommon disorder characterized by inflammation in the blood vessels, which can damage organ systems. The nose, sinuses, throat, lungs, and kidneys are commonly affected by GPA.
What are the Causes of Granulomatosis with Polyangiitis?
The exact causes of granulomatosis with polyangiitis are unknown; however, there is evidence to suggest that the immune system plays an important role. The condition can result in the formation of inflammatory tissue masses (granulomas) which destroy normal tissue, and restricted blood arteries reduce the amount of blood and oxygen that reaches the tissues and organs of your body.
One of the risk factors for granulomatosis with polyangiitis is that it affects people between the ages of 40 and 65.
What are the Symptoms of Granulomatosis with Polyangiitis?
Signs and symptoms of granulomatosis with polyangiitis may include:
- Pus-like drainage with crusts from your nose
- Sinus infections, and nosebleeds
- Coughing
- Shortness of breath or wheezing
- Fever
- Fatigue
- Joint pain
- Weight loss
- Blood in the urine
- Numbness in the limbs and fingers
- Skin sores, bruising, or rashes
- Eye redness and vision problems
- Ear inflammation and hearing problems
Diagnosis of Granulomatosis with Polyangiitis
Your doctor will review your medical history and symptoms and based on this a physical examination will be performed. Your doctor may also recommend the following diagnostic tests:
Lab Tests
- Blood tests are performed to check for signs of inflammation, anemia, anti-neutrophil cytoplasmic antibodies (who have active granulomatosis with polyangiitis)
- Urine testing is done to detect the presence of red blood cells, protein, or other abnormalities in the urine.
Imaging Tests
- Chest X-rays: This study uses electromagnetic beams to produce images of the chest and detect any inflammation.
- MRI Scan: This is an imaging study that uses a large magnetic field and radio waves to detect any damage to organs and soft tissues.
Biopsy
A small sample of tissue from the affected area is taken and observed under the microscope. A biopsy can confirm a diagnosis of granulomatosis with polyangiitis.
Treatment for Granulomatosis with Polyangiitis
Treatments for granulomatosis with polyangiitis include:
Medications
Prednisone and other corticosteroids assist to inhibit the immune system and reduce blood vessel inflammation.
Cyclophosphamide, azathioprine, mycophenolate, and methotrexate are some of the other immune-suppressing medicines that work to suppress your immune system. Another treatment option is rituximab, which is given by injection, and often is combined with corticosteroids.
Plasma exchange (Plasmapheresis)
This treatment eliminates the disease-causing chemicals from the liquid portion of your blood, known as plasma. You receive fresh plasma or the protein albumin, which allows your body to produce new plasma.
