Granulomatosis with Polyangiitis (Wegener's): Symptoms, Treatment

Overview

Granulomatosis with polyangiitis (Wegener’s), also called GPA, is an uncommon disease that causes inflammation in the blood vessels. Formerly, it is known as Wegener’s granulomatosis. It mainly affects the ears, nose, sinuses, lungs and kidneys, but it can also affect other organs. When the inflammation, granulomas in this condition, blocks the blood flow in the vessels, organ disability may occur.

The specific cause of GPA is unknown. It can affect anyone at any age, but white people of middle age, mostly between 40 and 65 years old, are more dangerous populations. Luckily, prompt diagnosis and treatment can lead to full recovery. If left untreated, however, GPA can be lethal.


Cause

The cause of GPA is unclear till now. It is not a contagious disease, but researches show that genes do play a role, minor though, in GPA. Other risk factors include region and age. More specifically, it most often affects white people between the ages of 40 and 65.


Symptoms

Early signs and symptoms of GPA are extremely different so diagnosis can be delayed. Different affected areas may present variant symptoms. GPA can cause:

  • General symptoms like fatigue, fever, weight loss, muscle and joint pain
  • Ear problems such as earache and hearing loss
  • Nose problems such as blocked nose, nosebleeds and sinusitis
  • Eye problems like double vision, redness and burning feelings
  • Lung problems, including cough, chest pain and shortness of breath
  • Kidney problems like blood in the urine, high blood pressure
  • Skin problems such as rashes and small red or purple spots
  • Gut problems, including diarrhea and blood in poo

If it’s not treated in time, GPA can result in permanent damage to some parts of the body. For example, it can cause hearing loss, change the shape of the nose or stop the kidneys from working properly. GPA may also lead to blood clot in one or more deep veins, especially in the leg.


Diagnosis

To make definite diagnosis, lab tests, imaging technologies and biopsy can help.

General lab tests for diagnosing GPA are:

An X-ray, CT scan or MRI scan can help doctors look at and examine affected parts of the body.

In a biopsy, your doctor will remove a small sample of tissue from an affected area and check it for signs of inflammation.


Treatment

Treatment options for GPA vary depending on severity of the condition. Treatment now is directed toward stopping the inflammation process by suppressing the immune system and reducing inflammation in the blood vessels. Medications used in this case include:

When your condition is improved after taking the two medications mentioned above, you can take some long-term drugs like Methotrexate, to prevent reoccurrence of GPA.

Another option for treating GPA is plasma exchange. Doctors will remove the liquid portion of your blood (plasma) containing substances associated with GPA. Then you receive fresh plasma or a protein made by the liver to produce new plasma. For patients who have very serious GPA, plasma exchange can help the kidneys recover.


Keywords: granulomatosis with polyangiitis; GPA.


Related Posts:

What is GPA?

What are the Basics of Granulomatosis with Polyangiitis?

What are the Common Signs of Wegener’s Granulomatosis?

What is the Life Expectancy of Wegener’s Disease?

* The Content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.