Granulomatosis with Polyangiitis (GPA)

Written by Akshara Koneru

Edited by Anirudh Koneru

Published on 8/26/2020

GPA stands for more than just your "grade point average": granulomatosis with polyangiitis ( GPA) is a rare disease in which blood vessels from all parts of the body experience swelling, resulting in damage to the respiratory system as well as the kidneys.

The first part of the name, granulomatosis, is derived from the formation of granulomas (small regions of inflammation that contain cells from the immune system) that are often found in the lungs as well as other parts of the respiratory tract. The second term, polyangiitis, is used to describe the multitude of types of blood vessels that experience swelling. GPA most often affects those with northern European ancestors. Every person who is affected by this disease has a different experience with it, whether it be how quickly it develops or the severity of its impact.

Signs and Symptoms

Initial symptoms of GPA can be mistaken for the common cold, such as runny nose, congestion, fatigue, coughing, and generally feeling ill. More severe symptoms include nosebleeds, swelling of the windpipe, joints, muscles, and sinuses, middle ear swelling (that can end up in hearing loss if untreated), and a collapse of the bridge of the nose (called saddle-nose).

Additionally, some may experience pulmonary symptoms (affecting the lungs), that may include coughing up blood, having trouble breathing, chest pain, inflammation of lung tissue, and bleeding of the lungs, which calls for hospitalization. Inflammation can be found in various parts of both the eye (which can result in loss of vision) as well as the heart (that may lead to strokes or heart attacks), though the latter is extremely rare. Parts of the skin may be affected as well, such as bumps on the skin or bleeding between layers of skin. Cold fingers or toes may occur due to the lack of blood flow to those areas, which are occasionally severe enough to result in the death of tissues of the fingers and toes. In the most extreme case, certain individuals may experience neurological issues that cause pain and weakness in the brain as well headaches and seizures.

Etiology

Unfortunately, the cause of GPA is not completely understood, but is thought to be an auto-immune disease. Auto-immune diseases occur when the immune system (responsible for protecting the body from infections and diseases) attacks the body’s own cells and tissues. The immune system uses antibodies (types of proteins) that attack diseases. Individuals with GPA often have specific antibodies known as antineutrophil cytoplasmic antibodies (ANCA) that result in the immune system attacking blood vessels.

This leads to the inflammation of those blood vessels, causing the various signs and symptoms of GPA. However, the reason as to why the immune system attacks the body’s blood vessels is not confirmed. Theories include environmental influences (such as diet and exercise) or simply being born with a higher chance of having an auto-immune disorder.

Diagnosis

GPA is diagnosed with a detailed process that begins with an in-depth research of patient history and observing and identifying present symptoms. If it is suspected that the disease is present, blood tests are done to detect inflammation and the existence of ANCA throughout the body. X-rays of the chest are also done in order to check for inflammation in the lungs.

If the results of those tests indicate GPA, removing and examining a piece of tissue from the body (a biopsy) can be done in order to detect inflammation of immune cells (granulomatosis) and swelling of blood vessels (vasculitis). Samples are most often taken from the lungs, sinuses and kidneys, but a lung biopsy typically produces the most accurate results. If it is established that an individual has GPA, further testing is important in order to determine what other areas in the body are affected by the disease. This includes testing of lung and kidney function in addition to an ocular (eye) exam.

Rarity

The rarity of GPA differs with each specific population that is studied. However, because this disease is often left undetected in many individuals, it is difficult for scientists and researchers to conclude the exact number of people who are affected by this disease.

Resources

There are many organizations and foundations that are available to help patients and caretakers who struggle with GPA or other auto-immune and rare diseases.

The American Autoimmune & Related Diseases Association Inc. (contact information and description can be found at http://www.arda.org/) and the Vasculitis Foundation (information found at https://www.vasculitisfoundation.org/) are two such resources for patients or advocates looking to learn more about GPA.

If you are looking for clinical testing opportunities, the following link has a multitude of studies that are looking to recruit patients: https://clinicaltrials.gov/ct2/results?cond=GPA&Search=Apply&recrs=a&age_v=&gndr=&type=&rslt=.

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Citations

Granulomatosis with polyangiitis. (2018, January 14). Retrieved August 16, 2020, from https://rarediseases.info.nih.gov/diseases/7880/granulomatosis-with-polyangiitis

Matteson, E., & NORD. (2020, June 24). Granulomatosis with Polyangiitis. Retrieved August 16, 2020, from https://rarediseases.org/rare-diseases/granulomatosis-with-polyangiitis/

James M. Steckelberg, M. (2018, September 26). Granuloma: What does it mean? Retrieved August 16, 2020, from https://www.mayoclinic.org/granuloma/expert-answers/faq-20057838

Mayo Clinic Staff. (2019, April 20). Common cold. Retrieved August 16, 2020, from https://www.mayoclinic.org/diseases-conditions/common-cold/symptoms-causes/syc-20351605