Wegener's Granulomatosis

Wegener's granulomatosis, also known as granulomatosis with polyangiitis (GPA), is a rare disease characterized by the inflammation of blood vessels. In addition to blocking blood flow to vital organs, this condition causes the formation of granulomas (inflamed masses) around the blood vessels, destroying normal tissue.

The condition may affect the ears, nose and sinuses. In the most serious cases, the kidneys, lungs and upper respiratory tract are commonly affected. Restricted blood flow to these areas can cause permanent damage. Although this disease can be fatal if left untreated, early diagnosis and treatment can lead to complete recovery.

Causes of Wegener's Granulomatosis

While the cause of Wegener's granulomatosis is unknown, research indicates that this autoimmune disorder is triggered by an event that results in inflammation. In some individuals, this inflammation is believed to set off an abnormal immune system reaction. The abnormal reaction of the immune system leads to further inflammation, constriction of the blood vessels, and the creation of granulomas. It is possible that an infection is the original trigger, but to date no specific infection has been linked to the condition.

Wegener's granulomatosis commonly presents in patients between the ages of 40 and 65, most often of northern European descent. It is rarely seen in children but has been documented in infants as young as 3 months of age.

Symptoms of Wegener's Granulomatosis

Frequent bouts of sinusitis, sometimes accompanied by fever, malaise, and night sweats, are a common symptom of Wegener's granulomatosis. Other symptoms may include:

Chronic runny nose
Cough
Hoarseness
Joint pain
Chronic ear infections
Skin changes
Conjunctivitis

Since these symptoms may also be the result of a number of other medical problems, they may not lead to a prompt diagnosis. It is the following, more serious, symptoms that typically bring patients in for medical consultation:

Nosebleeds, inflamed gums
Night sweats
Bloody cough, chest pain
Shortness of breath or wheezing
Blood in urine
Severe swelling of the eye

Patients with Wegener's may also suffer a loss of appetite and subsequent weight loss.

Diagnosis of Wegener's Granulomatosis

Diagnosing Wegener's granulomatosis typically involves a battery of tests. These tests may include:

Blood tests for certain proteins
Urinalysis to detect protein or blood in the urine
Chest X-rays
CT scan of the chest or sinuses
Bronchoscopy

A definitive diagnosis of Wegener's granulomatosis depends on the results of a biopsy of affected tissue. The tissue may be removed from the nasal mucosa, air passages or lungs, skin or kidneys. The biopsy is performed to determine whether either vasculitis or granulomas are present.

Treatment of Wegener's Granulomatosis

With early diagnosis and treatment, patients may recover from Wegener's granulomatosis within a few months, though they usually require maintenance therapy for up to 2 years or longer. Careful monitoring is necessary because the disease recurs in approximately half of all patients. Treatments for Wegener's granulomatosis include administration of:

Corticosteroids, such as prednisone
Immune suppressants, such as methotrexate
Biologic medications

Other prescribed drugs may be used to counteract the side effects, such as bone loss or lung infections, of these primary medications. Folic acid or folinic acid (leucovorin) are usually prescribed to combat the depletion of folate associated with methotrexate use.

Complications of Wegener's Granulomatosis Treatments

In severe cases, or cases detected during advanced stages, this disorder may have to be treated with experimental medications, such as intravenous immunoglobulin. If the kidneys have been damaged, a plasma exchange, or even a kidney transplant, may be necessary.

Ambreen Ashraf MD

Arthritis and Autoimmunity Clinic

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Ambreen Ashraf, MD

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