Wegener's granulomatosis
Definition:
Wegener's granulomatosis (VEG-eh-nerz gran-u-loe-muh-TOE-sis) is an uncommon disorder that causes inflammation of your blood vessels. This inflammation restricts blood flow to various organs.
Wegener's granulomatosis, which is also called granulomatosis with polyangiitis (GPA), often affects your kidneys, lungs and upper respiratory tract.
The restricted blood flow to these organs can damage them. Wegener's can affect other organs, but this isn't as common, and generally isn't as serious.
Wegener's granulomatosis also produces a type of inflammatory tissue known as a granuloma that's found around the blood vessels. Granulomas can destroy normal tissue. There is no known cause for Wegener's granulomatosis.
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.
Symptoms:
Signs and symptoms of Wegener's granulomatosis may develop suddenly or over several months.
The first Wegener's granulomatosis symptoms usually involve areas of your respiratory tract, such as your sinuses, throat or lungs. However, the condition often worsens rapidly, affecting blood vessels and the organs they supply, such as the kidneys. Signs and symptoms may include:
When to see a doctor
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 this disease can get worse quickly, early diagnosis is critical to receiving effective treatment.
Causes:
The cause of Wegener's granulomatosis is unknown. It appears to develop after an initial inflammation-causing event 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). The triggering event may be an infection, but no specific infection has been identified as the cause.
Complications:
Besides affecting 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:
With early diagnosis and appropriate treatment, you may recover from Wegener's granulomatosis within a few months. Maintenance therapy often needs to be continued for 18 to 24 months. In some cases, longer treatment may be necessary. Because the disease can recur, your doctor will continue monitoring your condition closely after treatment.
Medications
Your doctor may prescribe:
Side effect treatments
Because of potential side effects of the drugs used to treat Wegener's, such as lowering 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:
Definition:
Wegener's granulomatosis (VEG-eh-nerz gran-u-loe-muh-TOE-sis) is an uncommon disorder that causes inflammation of your blood vessels. This inflammation restricts blood flow to various organs.
Wegener's granulomatosis, which is also called granulomatosis with polyangiitis (GPA), often affects your kidneys, lungs and upper respiratory tract.
The restricted blood flow to these organs can damage them. Wegener's can affect other organs, but this isn't as common, and generally isn't as serious.
Wegener's granulomatosis also produces a type of inflammatory tissue known as a granuloma that's found around the blood vessels. Granulomas can destroy normal tissue. There is no known cause for Wegener's granulomatosis.
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.
Symptoms:
Signs and symptoms of Wegener's granulomatosis may develop suddenly or over several months.
The first Wegener's granulomatosis symptoms usually involve areas of your respiratory tract, such as your sinuses, throat or lungs. However, the condition often worsens rapidly, affecting blood vessels and the organs they supply, such as the kidneys. Signs and symptoms may include:
- Constantly runny nose, with pus-filled discharge
- Nosebleeds
- Sinus pain and inflammation (sinusitis)
- Ear infections
- Cough
- Chest pain
- Coughing up blood (hemoptysis)
- Shortness of breath
- General ill feeling (malaise)
- Unintended weight loss
- Joint aches and swelling
- Blood in urine (hematuria)
- Skin sores
- Eye redness, burning or pain
- Fever
When to see a doctor
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 this disease can get worse quickly, early diagnosis is critical to receiving effective treatment.
Causes:
The cause of Wegener's granulomatosis is unknown. It appears to develop after an initial inflammation-causing event 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). The triggering event may be an infection, but no specific infection has been identified as the cause.
Complications:
Besides affecting 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 a heart attack, though this is rare.
- 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.
With early diagnosis and appropriate treatment, you may recover from Wegener's granulomatosis within a few months. Maintenance therapy often needs to be continued for 18 to 24 months. In some cases, longer treatment may be necessary. Because the disease can recur, your doctor will continue monitoring your condition closely after treatment.
Medications
Your doctor may prescribe:
- Corticosteroids. Medications such as prednisone help suppress the immune system and treat the early signs and symptoms of Wegener's granulomatosis.
- Immune suppressant medications. Most people require another immunosuppressive drug, such as cyclophosphamide (Cytoxan), azathioprine (Azasan, Imuran) or methotrexate (Rheumatrex, Trexall), to counteract the body's immune reaction.
- Biologic therapy. Rituximab (Rituxan) is approved by the Food and Drug Administration for Wegener's. It reduces the number of a type of cell in your body (B cell) involved with inflammation. Rituximab may be as effective as cyclophosphamide in treating severe cases of Wegener's, and may be even better for treating Wegener's that recurs.
Side effect treatments
Because of potential side effects of the drugs used to treat Wegener's, such as lowering 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:
- Sulfamethoxazole-Trimethoprim (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
- Plasmapheresis. Also known as plasma exchange, this treatment removes the liquid portion of your blood (plasma) and separates it from the blood cells. The blood cells are then put back into your body, and your body produces new plasma to make up for what was removed. In people who have very serious Wegener's granulomatosis, plasmapheresis can help the kidneys recover.
- Surgery. If you've had kidney failure as a result of Wegener's granulomatosis, you may need a kidney transplant to restore normal kidney function. 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, in part, on how your other organs have been affected by the disease.
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