Wegener Granulomatosis is an inflammation of blood vessel that affects the lungs, kidney and other major organs of the body. If not taken proper prevention and treatment it is a life-threatening disease. However this requires a long term immune suppressant treatment. The disease was discovered in 1936 by the physician Dr. Friedrich Wegener. Reports states that Wegener's granulomatosis affects small and medium-sized vessels, it is formally classified as one of the small vessel vasculatures in the Chapel Hill system. Wegener's granulomatosis usually affects young or middle-aged adults. Although it is uncommon in children, it can affect people at any age.
Symptoms of Wegener Granulomatosis:
This syndrome usually shows no specific nature of symptoms. Generally the patients show the first signs of Rhinitis. Other progressive symptoms are:
- Rapidly progressive glomerulonephritis
- Nose bleeding, loss of hearing,
- Underlying bone destruction and loosening of teeth
- Conjunctivitis
- bronchial stenosis
- Arthritis
- Feeling fatigue and weight loss along with fevers
- shortness of breath
- skin nodules or ulcers
- Chest pain
- Cough, with or without blood
- Voice change, wheezing, or shortness of breath caused by inflammation of the trachea
Other possible symptoms include:
- Eye inflammation or bulging, with or without loss of vision
- muscle pain
- Rashes or skin sores
- Kidney inflammation
Causes:
The major cause of Wegener's granulomatosis is not known. Wegener's granulomatosis is a rare disorder in which blood vessels become inflamed, making it hard for blood to flow. It is thought to be an autoimmune disorder. Wegener granulomatosis is typically suspected only when a patient has had baffling symptoms for a long period of time. Lab findings of patients with Wegener's granulomatosis include urine tests that detect protein and red blood cells in their urine and x-ray tests of the chest and sinuses detects abnormalities resulting from lung and sinus inflammation
To the presence of Wegener granulomatosis your doctor may suggest one of the following:
- Bronchoscopy with biopsy
- Kidney biopsy
- Nasal mucosal biopsy
- Open lung biopsy
- Skin biopsy
- Upper airway biopsy
Other tests that may be done include:
- Bone marrow aspiration
- Chest CT scan
- Chest x-ray
Treatment:
Medicines used to treat Wegener's granulomatosis include:
- Azathioprine
- Cyclophosphamide
- Methotrexate
The critical part of these medicines are that they may cause serious side effects and it is recommended that you should carefully discuss your treatment plan with your doctor, before intake of these medicines.
Other medicines may be prescribed, including:
- Bisphosphonate to prevent bone loss caused by prednisone
- Folic acid or folinic acid
- methotrexate Trimethoprim to prevent lung infections
- Glucocorticoid drugs
- Cytotoxic drugs
Some patients do require a low dose of prednisone to sustain remission. The ideal duration of treatment with the other immunosuppressive medications is uncertain. Indeed many patients who have milder forms of WG can be treated without cyclophosphamide; being able to achieve remission with agents such as methotrexate, mycophenolate mofetil or rituximab. The treatment used for Wegener Granulomatosis has also been successfully applied to other vasculitic diseases.
However, due to the increased risk of developing serious infections, these drugs suppress the immune systems and can also result in reactions like eight gain, cataracts, brittle bones, high blood pressure, diabetes, and changes in mood and personality. Because the medicines used to treat WG can have serious side effects, patients are monitored closely by their doctor. The dosage of medicine is adjusted as needed throughout the course of treatment.
