Understanding Granulomatosis With Polyangiitis Symptoms

You may occasionally hear GPA called Wegener's granulomatosis. This is because the disease was initially called Wegener’s arteritis, Wegener’s disease, or Wegener's granulomatosis. It was named after a German pathologist, Friedrich Wegener, who had three cases and recognized it as a different form of vasculitis. The name was changed to Granulomatosis with Polyangiitis or even GPA to remove any association with Wegener who was a Nazi war criminal.

Granulomatosis with Polyangiitis can affect several parts of your body. It commonly affects the respiratory system, including:

• Nose
• Sinuses
• Throat
• Lungs

It can also affect other parts of your body including:

• Kidneys
• Eyes
• Heart
• Skin
• Ears
• Fingers
• Toes

Sometimes it will only affect one part of your body, such as just the lungs or kidneys.

Granulomatosis with Polyangiitis has a number of common symptoms. They include:

• Blood in urine
• Bloody phlegm
• Coughing
• Ear infections
• Eye redness, pain, or burning
• Fatigue
• Fever
• General aches and pains
• Joint pain
• Nosebleeds
• Numbness in your fingers, toes, or limbs
• A runny nose
• Shortness of breath
• Sinus infections
• Skin sores or bruising
• Stuffy nose
• Weight loss
• Wheezing

Granulomatosis with Polyangiitis can cause the following complications without treatment:

• Deep vein thrombosis
• Hearing loss
• Heart disease
• Kidney damage
• Weakened cartilage in the nose resulting in the collapse of the bridge of the nose

Some of these complications can be life-threatening, which is why if you have any of the above symptoms mentioned, you should see a doctor about getting treated.

Granulomatosis with Polyangiitis in the inflammation of the arteries and veins. It typically affects the arteries that provide a blood supply to the kidneys, lungs, and sinuses. Sometimes it only affects one organ, such as the lungs. This is an incomplete form of the disease. The inflammation is in the form of granulomas that can severely damage the organs.

Unfortunately, doctors do not know what precisely causes Granulomatosis with Polyangiitis. They know that usually occurs after an infection, but no particular infection is a culprit. There may or may not be a small genetic component to it, but it is rare for two members of the same family to have it. Although it typically affects people between the ages of 40 and 65, it can affect anyone at any age.

Your doctor will examine your and go through your symptoms. If they suspect Granulomatosis with Polyangiitis, they will need to run several tests to determine if you have the disease. This includes the biopsy of the trachea, sinuses, nose, and ears. Not as invasive but is less than 50 percent successful at diagnosis.

The best way to prove there is Granulomatosis with Polyangiitis is a lung biopsy, although a kidney biopsy may be used as well. These biopsies, while invasive, are usually able to confirm the diagnosis. The lung biopsy may be done thoracoscopic or open. The kidney biopsy usually requires a smaller tissue sample.

There are several less invasive ways to diagnose Granulomatosis with Polyangiitis. These include:

• Blood tests -- Blood tests can look for inflammation, antibodies (ANCA tests), and anemia.
• Chest images -- MRI and X-rays can show how the organs and blood vessels have been affected.
• Urine tests -- Urine tests can determine if there is blood in your urine or if there is any damage to your kidneys.

Treatment of Granulomatosis with Polyangiitis is aimed at reducing inflammation and suppression of the immune system which reacts to the inflammation with more inflammation. Medications include:

• Corticosteroids to reduce inflammation
• Antibiotics
• Immunosuppressors

About 90 percent of patients respond to treatment with 75 percent having remission. However, there is a high relapse rate which means that those who have Granulomatosis with Polyangiitis will need to continue with medications to prevent relapse.