Wegener's granulomatosis is an uncommon disorder that causes inflammation of your blood vessels, which in turn restricts blood flow to various organs. Most commonly Wegener's granulomatosis affects your kidneys, lungs and upper respiratory tract. The restricted blood flow to these organs can damage them. Besides inflamed blood vessels, Wegener's granulomatosis produces a type of inflammatory tissue known as granuloma, found around the blood vessels. Granulomas can destroy normal tissue. What causes Wegener's granulomatosis is uncertain, but it's not an infection nor is it a type of cancer. Early diagnosis and treatment of Wegener's granulomatosis may lead to a full recovery. Without treatment, Wegener's granulomatosis can be fatal, most commonly from kidney failure.

Complications of Wegener’s Granulomatosis

Besides your upper and lower respiratory tracts, Wegener's granulomatosis may affect other organs, including your skin, eyes, ears, kidneys, spinal cord and heart. Complications may include:

• Hearing loss. Inflammation of granulated tissue in the middle ear can lead to hearing loss.
• Skin scarring. Sores may develop and may lead to severe scarring.
• Heart attack. Wegener's granulomatosis can affect the arteries of your heart, resulting in chest pain or heart attack.
• Kidney (renal) damage. As Wegener's granulomatosis progresses, it's likely to affect your kidneys. It can lead to glomerulonephritis, a type of kidney disease that hampers your kidneys' ability to remove waste and excess fluid from your body, leading to a buildup of waste products in your bloodstream (uremia). Kidney failure is a leading cause of death from Wegener's granulomatosis.

Causes of Wegener’s Granulomatosis

The cause of Wegener's granulomatosis is unknown, but it appears to develop as a result of an initial inflammation-causing event that triggers an abnormal reaction from your immune system. The combination of these events can lead to inflamed, constricted blood vessels and harmful inflammatory tissue masses (granulomas). Scientists speculate that the initial event may be an infection. This has been documented in nearly half the people with Wegener's, but the infections aren't overwhelming. Other possible contributing causes include an environmental toxin, a genetic predisposition to the condition or even a combination of all of these. This question has yet to be resolved.

Signs & Symptoms of Wegener’s Granulomatosis

Signs and symptoms of Wegener's granulomatosis symptoms may develop suddenly or gradually. The first Wegener's granulomatosis symptoms usually involve your respiratory tract. However, the condition often worsens rapidly, affecting blood vessels and the organs they supply. Signs and symptoms may include:

• Constantly runny nose
• Nosebleeds
• Sinus pain and inflammation (sinusitis)
• Ear infections
• Cough
• Coughing up blood
• Shortness of breath
• General ill feeling (malaise)
• Appetite loss
• Joint aches and swelling
• Skin sores
• Eye redness, burning or pain
• Double or decreased vision
• Fever
• Weakness and fatigue, possibly associated with low red blood cell count (anemia)

For some people, the disease limits itself to the respiratory tract and doesn't involve the kidneys. Kidney involvement usually doesn't cause symptoms early in the course of the disease, and may be detected only by blood and urine tests. However, in time kidney failure with its associated anemia occurs.

Diagnosis of Wegener’s Granulomatosis

Besides asking you about your signs and symptoms, conducting a physical exam and taking a medical history, your doctor may request several tests, including:

• Blood tests. If your doctor suspects Wegener's granulomatosis, he or she may order a blood test that can detect certain proteins (autoantibodies) in your blood called anti-neutrophil cytoplasmic autoantibodies (ANCA). These autoantibodies appear in the blood of 80 to 95 percent of people with active Wegener's granulomatosis. The presence of these autoantibodies lends support to a diagnosis of Wegener's granulomatosis, but isn't enough to confirm that you have the disease. Your blood test also can measure your erythrocyte sedimentation rate — commonly referred to as sed rate. By measuring how quickly red blood cells fall to the bottom of a tube of your blood over a period of one hour, this test may indicate the level of inflammation in your body. Generally, red blood cells fall faster when inflammation is present. Another blood test can check for anemia, which is common in people with this disease. A blood test for creatinine will assess if your kidneys aren't properly filtering waste products from your blood.
• Urine tests. These evaluate kidney function to determine whether the disease is affecting your kidneys.
• Chest X-ray. This test shows cavities or masses in your lungs. However, it can't distinguish between Wegener's granulomatosis and other lung diseases.
• Biopsy of affected tissue. The only sure way to confirm a diagnosis of Wegener's granulomatosis is to remove a small piece of tissue from an affected organ (biopsy) and examine it under a microscope. Your doctor may remove tissue from your nasal passages, airways or lungs to confirm or rule out the presence of both vasculitis and granulomas. Other areas for biopsy may include your skin or your kidneys. Some biopsies can be performed in an outpatient setting using a numbing medication (local anesthetic). Others, such as an open-lung biopsy, may require hospitalization.

Treatments of Wegener’s Granulomatosis

With early diagnosis and appropriate treatment, you may recover from Wegener's granulomatosis within a few months. In some cases, longer treatment may be necessary. Because the disease can recur, your doctor will continue monitoring your condition after treatment.

Medications

Your doctor may prescribe corticosteroids, such as prednisone, to treat the early signs and symptoms of Wegener's granulomatosis. For some people, a corticosteroid may be enough. However, most people require another immunosuppressive drug, such as cyclophosphamide (Cytoxan), azathioprine (Imuran) or methotrexate (Rheumatrex), to counteract the body's immune reaction. When standard treatments aren't effective, some doctors who are experienced in treating Wegener's are using experimental drugs. One such drug is rituximab (Rituxan), which reduces the number of a type of cell in your body (B cell) that's involved with inflammation. But further studies are needed to see if this drug should be considered standard treatment for Wegener's. Rituxan was developed to treat a type of cancer known as non-Hodgkin's lymphoma and has since been approved for the treatment of rheumatoid arthritis. Because of potential side effects of the drugs used to treat Wegener's, such as hampering your body's ability to fight off infection, your doctor will monitor your condition while you're taking them. Drugs your doctor may prescribe to help prevent drug-related side effects include:

• Trimethoprim-sulfamethoxazole (Bactrim, Septra) to prevent lung infection
• Medicine such as bisphosphonates (Fosamax) to prevent bone loss (osteoporosis) associated with prednisone use
• Folic acid, a synthetic form of the B vitamin folate, to prevent sores and other signs and symptoms associated with the depletion of folate in your body from methotrexate use

Surgery

If you've had kidney failure as a result of Wegener's granulomatosis, you may want to consider a kidney transplant to restore normal kidney function. A successful transplant depends on finding the organ that minimizes the chances your body will reject it and following a treatment regimen that will allow your body to accept the new organ. Talk to your doctor to determine whether a kidney transplant might be an option for you. Your eligibility to receive a donated kidney will depend on how your other organs have been affected by the disease.

When to seek Medical Advice

See your doctor if you have a persistently runny nose that doesn't respond to over-the-counter (OTC) cold medicines, especially if it's accompanied by nosebleeds and pus-like material, coughing up blood, or other possible signs and symptoms of Wegener's granulomatosis. Because the disease can progress quickly, early diagnosis is critical to receiving effective treatment.