Granulomatosis with polyangiitis

Granulomatosis with polyangiitis (GPA) is a rare disorder in which blood vessels become inflamed. This leads to damage in major organs of the body. It was formerly known as Wegener's granulomatosis.

Causes

GPA mainly affects blood vessels in the nose, sinuses, ears, lungs, and kidneys. Other areas may also be affected in some cases. The disease can be severe and prompt treatment is important.

The exact cause is unknown, but it is an autoimmune disorder .

Autoimmune disorder

An autoimmune disorder occurs when the body's immune system attacks and destroys healthy body tissue by mistake. There are more than 80 types of aut...

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GPA is most common in middle-aged adults of northern European descent. It is rare in children, but has been seen in infants as young as 3 months old.

Symptoms

Frequent sinusitis is the most common symptom. Other early symptoms include a fever that has no clear cause, night sweats, fatigue , and a general ill feeling ( malaise ).

Other common symptoms may include:

• Chronic ear infections
• Respiratory such as nose bleeds, pain, and sores around the opening of the nose
• Cough with or without blood in the sputum .
• Chest pain and shortness of breath as the disease progresses
• Loss of appetite and weight loss
• Skin changes such as bruises and ulcers of the skin
• Kidney problems
• Bloody urine
• Eye problems ranging from mild conjunctivitis to severe swelling of the eye.

  Conjunctivitis

  The conjunctiva is a clear layer of tissue lining the eyelids and covering the white of the eye. Conjunctivitis occurs when the  conjunctiva becomes...

  

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Less common symptoms include:

• Joint pain
• Weakness
• Abdominal pain

Exams and Tests

You may have a blood test that looks for proteins called antineutrophil cytoplasmic antibodies (ANCA). These are often found in people with active GPA. However, this test is sometimes comes back negative even in people with the condition.

A chest x-ray will be done to look for signs of lung disease.

Urinalysis is done to look for signs of kidney disease such as protein and blood in the urine. Sometimes urine is collected over 24 hours to check how the kidneys are working.

Urinalysis

Urinalysis is the physical, chemical, and microscopic examination of urine. It involves a number of tests to detect and measure various compounds th...

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A biopsy is sometimes needed to confirm the diagnosis. The exact type of biopsy depends on which area of the body the doctor wants to look at. You may have one of the following:

• Bronchoscopy with biopsy of the lung
• Kidney biopsy
• Nasal mucosal biopsy

  Nasal mucosal biopsy

  A nasal mucosal biopsy is the removal of a small piece of tissue from the lining of the nose so that it can be checked for disease.

  

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• Open lung biopsy

  Open lung biopsy

  An open lung biopsy is surgery to remove a small piece of tissue from the lung. The sample is then examined for cancer, infection, or lung disease....

  

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• Skin biopsy
• Upper airway biopsy

  Upper airway biopsy

  Upper airway biopsy is surgery to remove a small piece of tissue from the nose, mouth, and throat area for examination.

  

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Other tests that may be done include:

• Sinus CT scan

  Sinus CT scan

  Bone marrow is the soft tissue inside bones that helps form blood cells. It is found in the hollow part of most bones. Bone marrow aspiration is th...

  

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• Chest CT scan

Treatment

You may be treated glucocorticoids (such as prednisone). These are given along with other medicines that slow down the immune response.

Medicines used with prednisone to treat GPA include:

• Rituximab (Rituxan)
• Azathioprine (Imuran)
• Cyclophosphamide (Cytoxan)
• Methotrexate

These medicines are effective in severe disease but they may cause serious side effects. Talk to your health care provider about your treatment plan.

Other medicines used for the condition include:

• Medicines to prevent bone loss caused by prednisone
• Folic acid or folinic acid, if you are taking methotrexate
• Antibiotics to prevent lung infections

Support Groups

Support groups with others who suffer from similar diseases may help people with the condition and their families learn about the diseases and adjust to the changes associated with the treatment.

Outlook (Prognosis)

Without treatment, people with severe forms of this disease can die within a few months.

With treatment, the outlook for most patients is good. Most people who receive corticosteroids and other medicines that slow the immune response get much better.

The disease may return about half the time. It often comes back within 2 years of stopping treatment, so ongoing follow-up is needed.

Possible Complications

Complications most often occur when the disease is not treated. People with GPA develop tissue damage in the lungs, airways, and the kidneys. Kidney involvement may result in blood in the urine and kidney failure . Kidney disease can quickly get worse. Kidney function may not improve even when the condition is controlled by medicines.

Kidney failure

Acute kidney failure is the rapid (less than 2 days) loss of your kidneys' ability to remove waste and help balance fluids and electrolytes in your b...

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If untreated, kidney failure and possibly death occur in most cases.

Other complications may include:

• Eye swelling
• Lung failure
• Coughing up blood
• Nasal septum perforation (hole inside the nose)
• Side effects from medicines used to treat the disease

When to Contact a Medical Professional

Call your health care provider if:

• You develop chest pain.
• You cough up blood.
• You have blood in your urine.
• You have other symptoms of this disorder.

Prevention

There is no known prevention.