Granulomatosis with polyangiitis

Brief summary

• Granulomatosis with polyangiitis (GPA), also known as Wegener's ganulomatosis, is a type of vasculitis or swelling (inflammation) of the blood vessels.
• The disease can cause swelling of the blood vessels anywhere in the body but mainly impacts the sinuses, nose, trachea (windpipe), lungs, and kidneys.
• The swelling can limit the flow of blood to these body parts, causing damage.
• Symptoms of the disease can include sinus pain, recurrent respiratory infections, joint pain, tiredness (fatigue), and skin lesions.
• The exact cause of GPA is unknown, but it is a type of autoimmune disease.
• Diagnosis of GPA can be made with laboratory tests such as a blood test, biopsy of affected areas, and imaging of the lungs.
• Treatment of GPA often includes medications such as glucocorticoids and immunosuppressants.

What are the symptoms of granulomatosis with polyangitis?

• Granulomatosis with polyangiitis (GPA) can affect the blood vessels in any part of the body, but the most commonly affected areas include the sinuses, trachea, lungs, and kidneys.
• Granulomatosis with polyangiitis is the term used to describe this disease because people with this disease may have granulomas, which are areas of swelling that contain cells of the immune system.
• These granulomas are especially common in the lungs and airways of people with GPA. The term “polyangiitis” refers to swelling of many different types of blood vessels.

What are the signs of Granulomatosis with polyangiitis?

• The first sign of GPA may be a recurrent respiratory infection, or a cough or runny nose that continues for longer than expected.
• Other common symptoms of the disease include nosebleeds, joint pain, weakness, tiredness (fatigue), weight loss, or an unexplained fever.
• In some cases, the disease can cause the bridge of the nose to collapse, resulting in a saddle-nose deformity.
• Some people with GPA may have blood in the urine, chest pain, or skin lesions.
• If the disease is not treated, symptoms can progress to include kidney failure.
• More rarely, people with GPA may have symptoms affecting the eyes such as a recurrent eye infection or swelling of the eyes.
• Most people who have GPA start to have signs and symptoms of the disease in adulthood.
• The disease is most common in people of northern European descent.

Some of the common signs and symptoms include:

• Abnormal oral cavity morphology
• Joint pain
• Autoimmune disease
• Cerebral ischemia
• Bloody nose

What causes Granulomatosis with polyangiitis?

• 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.

How is Granulomatosis with polyangiitis 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.

How is Granulomatosis with polyangiitis 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:
• Blood test to determine if there are signs of swelling (inflammation) in the body
• Blood test for the presence of antineutrophil cytoplasmic antibodies (ANCA)
• Chest x-ray or CT scan of the lungs to determine if there is inflammation
• 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.

How is Granulomatosis with polyangiitis treated?

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

• Glucocorticoids such as prednisone
• Immunosuppressive drugs such as rituximab
• 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:
• Bisphosphonates to prevent bone weakening that can be caused by prednisone
• Antibiotics to prevent lung infections

What are the FDA-Approved treatments for this disease?

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

• Rituximab (Brand name: Rituxan) - Manufactured by Genentech, Inc.
• FDA-approved indication: For the use of Rituxan (rituximab) in combination with glucocorticoids for the treatment of pateints with Wegener's Granulomatosis (WG) and Microscopic Polyangiitis (MPA).

What is the prognosis of Granulomatosis with polyangiitis?

• 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.

How long can you live with GPA?

If left untreated, severe GPA is associated with a very high (>90%) mortality rate with a mean survival of 5 months from diagnosis(with modern treatment, the median survial is much higher, 80% or more of patients living after 8 years.

How long can you live with Wegener's granulomatosis?

• With proper treatment, more than 80% of treated patients are alive at least eight years later.
• How serious is granulomatosis with Polyangiitis?
• Early diagnosis and treatment of granulomatosis with polyangiitis is very important as it may lead to a full recovery.
• However, without treatment, the condition can be fatal.

Is Wegener's granulomatosis a terminal illness?

Wegener's granulomatosis may be fatal without proper diagnosis and prompt medical treatment.

What foods should I avoid with vasculitis?

You should aim to cut down on starchy foods such as bread, potatoes, rice and pasta, replacing these with fresh fruit and vegetables. You should also avoid processed food and grain fed meat.

What triggers Wegener's disease?

While the cause of Wegener's granulomatosis is unknown

Can you live a long life with ANCA vasculitis?

Yes, with proper treatment. Life expectancy during past 15 years for AAV patients increased from 99.4 to 126.6 months. A high BVAS score at the onset of the disease is a bad prognostic factor related to shorter life expectancy.

Does Wegener's run in families?

The cause of granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is currently unknown.

Can Wegener's affect the eyes?

Wegener's granulomatosis (WG) is a systemic disease with obscure aetiology that can affect the eye.

What is the prognosis for Wegener's disease?

Eighty-eight percent of patients survived the first year follow-up since the diagnosis, while 84% of patients remained alive after the second year of observation

FAQS

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• Does Wegener's disease cause pain?
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