Granulomatosis with polyangiitis

What causes granulomatosis with polyangiitis (GPA)?

The exact cause of granulomatosis with polyangiitis (GPA) is not well- understood. It is thought that GPA is an autoimmune disease. Autoimmune diseases occur when the immune system, which is responsible for protecting the body from infection, mistakenly attacks tissues of the body. The immune system is supposed to release disease- fighting proteins called antibodies that attack potential sources of infection. People who have GPA frequently have specific antibodies called antineutrophil cytoplasmic antibodies (ANCA). These antibodies cause the immune system to attack the blood vessels. This causes swelling (inflammation) of blood vessels, which causes the signs and symptoms of GPA.

Exactly what causes the immune system to mistakenly attack the blood vessels is not clear. In general, it is thought that autoimmune diseases are caused by a combination of being born with an increased likelihood to develop the disease (genetic predisposition) as well as exposure to environmental factors. It is not known exactly what environmental factors may be associated with GPA, but it may be that having a previous infection can increase the risk to develop the disease.

Last updated on 05-01-20

How is granulomatosis with polyangiitis (GPA) diagnosed?

Granulomatosis with polyangiitis (GPA) is suspected when a doctor observes signs and symptoms that are consistent with the disease. These signs and symptoms often include a recurrent respiratory infection, tiredness (fatigue), weight loss, and unexplained fevers. Laboratory tests may then be ordered to confirm the diagnosis and rule out other causes of the symptoms. These tests may include:

Although none of these tests can be used by themselves to determine if a person has GPA, the tests can be used together to support a diagnosis of the disease. If GPA is suspected, a biopsy (removing a small piece of tissue) can be done to determine if the tissue shows granulomatosis (inflammation involving the immune cells) and vasculitis (swelling of the blood vessels). The part of the body from which a biopsy is taken is most commonly the lungs, sinuses, or kidneys.

Once a diagnosis of GPA has been confirmed, further tests may be necessary to determine if other parts of the body are affected. Tests of kidney or lung function may be completed, and an eye exam may be recommended.

Last updated on 05-01-20

Is granulomatosis with polyangiitis (GPA) inherited?

Changes (also known as pathogenic variants or mutations) in a particular gene are not known to be associated with granulomatosis with polyangiitis (GPA). In most cases, a person who has GPA is the only person in his or her family who has the disease. Therefore, it is not thought that GPA is passed directly from parent to child.

In some cases, instances of multiple people within the same family having GPA have been reported. It is thought that these cases may be caused by family members having normal variants in certain genes, such as the HLA- DPB1 gene, that increase the risk to develop GPA. However, having these normal variants does not guarantee that a person will develop GPA. It is thought that genetic changes in combination with environmental factors cause most cases of the disease.

Last updated on 05-01-20

What is the long-term outlook for people affected with granulomatosis with polyangiitis (GPA)?

The long-term outlook for people with granulomatosis with polyangiitis (GPA) may depend on how early an individual is diagnosed with the disease after the onset of symptoms, the severity of symptoms, and the response to treatment. For most people, the symptoms of GPA improve with treatment. In cases where a person has severe GPA that is not diagnosed or treated, there is a very high risk of death that can be caused by infection, respiratory failure (when too little oxygen passes from the lungs to the blood), kidney failure, and damage to the heart.

Most people with GPA receive treatment for about 18 months after they begin to recover from the disease. Receiving treatment for this length of time is aimed at preventing the symptoms from returning (relapse). However, about half of people with GPA may experience a return of the disease. This usually occurs within two years of stopping treatment. Therefore, it is extremely important that people with GPA continue to see their healthcare professionals regularly, both while receiving treatment and after stopping the medications.

After starting treatment for GPA, most people notice that their symptoms improve. However, the medications that help treat GPA can also cause serious side-effects such as bone weakening and having an immune system that is less able to fight off infection. Doctors may recommend additional medications that can help treat these side-effects.

Last updated on 05-01-20

How might granulomatosis with polyangiitis (GPA) be treated?

The treatment for granulomatosis with polyangiitis (GPA) usually includes a combination of multiple medications. These medications typically include:

If there are skin lesions present, they may be treated with a topical steroid that is applied to the affected area. Surgery may be recommended if swelling of the airway or lungs is preventing normal breathing.

Other medications may be recommended to help treat GPA. These medications are often used to treat the side-effects that may be associated with glucocorticoids and immunosuppressive drugs and may include:

Medscape Reference also provides information about the treatment of GPA, including a section detailing alternative and/or promising therapies.

Last updated on 05-01-20

Name: National Kidney Foundation 30 East 33rd Street
New York, NY, 10016, United States
Phone: 212-889-2210 Toll Free: 800-622-9010 Fax : 212-689-9261 Email: Url:
Name: Vasculitis Foundation P.O. Box 28660
Kansas City, MO, 64188, United States
Phone: +1-816-436-8211 Toll Free: 1-800-277-9474 Fax : +1-816-656-3838 Email: Url:
Name: American Autoimmune Related Diseases Association (AARDA) 22100 Gratiot Avenue
Eastpointe, MI, 48021, United States
Phone: 586-776-3900 Toll Free: 800-598-4668 Fax : 586-776-3903 Email: Url:

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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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