What is Granulomatosis with Polyangiitis?

Posted Tuesday, December 20, 2022

Did you know? GPA used to be known as Wegener’s granulomatosis after the first physician to describe the disease in medical literature in 1930.

Granulomatosis with polyangiitis most often affects people between the ages of 40 and 60.

01 | Introduction

Granulomatosis with polyangiitis (GPA) is an uncommon disorder where blood vessels in the nose, sinuses, throat, lungs and kidneys can become inflamed. This inflammation slows blood flow and can lead to damage in major organs. GPA is most common in adults and older people. Through early diagnosis and treatment, those with GPA can lead to a full recovery, but without treatment this condition can be fatal.

02 | Causes

The exact cause of GPA is unknown. This condition is not contagious and is not considered to be an inherited disorder. However, people with GPA may have a gene that makes them more likely to get the condition. This condition is most likely due to problems in the immune system, which cause it to attack itself and results in inflamed blood vessels.

03 | Symptoms

GPA can cause a variety of symptoms depending on which parts of the body are affected. The first signs involve the sinuses, throat or lungs and often worsens rapidly. Some symptoms may include:

  • Shortness of breath or wheezing
  • Joint pain
  • Weight loss
  • Persistent runny nose
  • Formation of nasal crusts or sores
  • Nose bleeds
  • Blood in the urine

04 | Treatments

GPA is treated with medicines that reduce the activity of the immune system. There are 3 main stages of treatment:

  • Stage 1: Bring the condition under control. This stage lasts a few months until the symptoms are under control.
  • Stage 2: Keeping the condition under control. This stage aims to stop symptoms from coming back and can last for 2 to 5 years.
  • Stage 3: Treating symptoms if they come back. If symptoms come back or new ones appear treatment may be changed or restarted.

Learn More

For more information on GPA and supportive resources, please visit mayoclinic.org.

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References

https://my.clevelandclinic.org/health/diseases/4757-granulomatosis-with-polyangiitis-gpa-formerly-called-wegeners 

https://www.nhs.uk/conditions/granulomatosis-with-polyangiitis/ 

https://www.pennmedicine.org/for-patients-and-visitors/patient-information/conditions-treated-a-to-z/granulomatosis-with-polyangiitis 

https://rarediseases.org/rare-diseases/granulomatosis-with-polyangiitis/

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