About pulmonary wegener's granulomatosis
What is pulmonary wegener's granulomatosis?
Lymphomatoid granulomatosis is a rare disorder characterized by overproduction (proliferation) of white blood cells called lymphocytes (lymphoproliferative disorder). The abnormal cells infiltrate and accumulate (form lesions or nodules) within tissues. The lesions or nodules damage or destroy the blood vessels within these tissues. The lungs are most commonly affected in lymphomatoid granulomatosis. Symptoms often include cough, shortness of breath (dyspnea) and chest tightness. Other areas of the body such as the skin, kidneys or central nervous system are also frequently affected.
The abnormal cells in lymphomatoid granulomatosis are B-cells (B lymphocytes) containing the Epstein-Barr virus. There are two main types of lymphocytes: B-lymphocytes, which may produce specific antibodies to "neutralize" certain invading microorganisms, and T-lymphocytes, which may directly destroy microorganisms or assist in the activities of other lymphocytes. Because lymphomatoid granulomatosis is caused by the overproduction of abnormal B-cells, affected individuals may eventually develop B-cell lymphoma, a form of non-Hodgkin lymphoma. Lymphoma is a general term for cancer of the lymphatic system.
What are the symptoms for pulmonary wegener's granulomatosis?
Signs and symptoms of granulomatosis with polyangiitis might include:
- Pus-like drainage with crusts from your nose, stuffiness, sinus infections and nosebleeds
- Coughing, sometimes with bloody phlegm
- Shortness of breath or wheezing
- Joint pain
- Numbness in your limbs, fingers or toes
- Weight loss
- Blood in your urine
- Skin sores, Bruising or rashes
- Eye redness, burning or pain, and vision problems
- Ear inflammation and hearing problems
For some people, the disease affects only the lungs. When the kidneys are affected, blood and urine tests can detect the problem. Without treatment, kidney or lung failure can occur.
What are the causes for pulmonary wegener's granulomatosis?
The cause of granulomatosis with polyangiitis isn't known. It's not contagious, and there's no evidence that it's inherited.
The condition can lead to inflamed, narrowed blood vessels and harmful inflammatory tissue masses (granulomas). Granulomas can destroy normal tissue, and narrowed blood vessels reduce the amount of blood and oxygen that reaches your body's tissues and organs.
What are the treatments for pulmonary wegener's granulomatosis?
With early diagnosis and appropriate treatment, you might recover from granulomatosis with polyangiitis within a few months. Treatment might involve taking prescription drugs long term to prevent relapse. Even if you're able to stop treatment, you'll need to regularly see your doctor — and possibly several doctors, depending on which organs are affected — to monitor your condition.Medications
Corticosteroids such as prednisone help suppress the immune system and reduce inflammation of the blood vessels. Common side effects include weight gain, risk of infection and osteoporosis.
Other drugs that suppress your immune system include cyclophosphamide, azathioprine (Azasan, Imuran), mycophenolate (CellCept) and methotrexate (Trexall). Rituximab (Rituxan) is another option for treating granulomatosis with polyangiitis. It's given by injection, and often is combined with corticosteroids.
Once your condition is controlled, you might remain on some drugs long term to prevent relapse. These include rituximab, methotrexate, azathioprine and mycophenolate.
Side effects of immune-suppressing drugs include increased risk of infection. Cyclophosphamide can cause nausea, diarrhea and hair loss. Your doctor may prescribe other drugs to help prevent side effects from prescribed treatments.Plasma exchange
Also known as plasmapheresis, this treatment removes the liquid portion of your blood (plasma) that contains disease-producing substances. You receive fresh plasma or a protein made by the liver (albumin), which allows your body to produce new plasma. In people who have very serious granulomatosis with polyangiitis, plasmapheresis can help the kidneys recover.More Information
- Kidney transplant
What are the risk factors for pulmonary wegener's granulomatosis?
Granulomatosis with polyangiitis can occur at any age. It most often affects people between the ages of 40 and 65.