Wegener's granulomatosis (Wegener's disease, granulomatosis with polyangiitis) - quite a rare form of vasculitis.It is believed to be an autoimmune disease in which most suffer the endothelial cells lining inside blood vessels.Wegener's granulomatosis - multisystem disease that can affect almost all organs.Most often marked pathological changes in the upper respiratory tract (almost 100%), lung (in the overwhelming number of patients) and kidney (75% of cases).Quite often affected joints (up to 50% of cases), and the eyes and skin.If a person is not involved in the process of the kidneys, the brain and the gut, it is believed that he is sick of "limited" form of Wegener's granulomatosis.However, even in this form of granulomatosis polyangiitis can occur very hard. Content
granulomatosis with polyangiitis- a rare disease that affects men and women of all ages.In most cases the disease is diagnosed in people over 40 years old.
is unknown what triggers the autoimmune mechanism of disease Wegener.It is believed that inflammation in the body or that could lead to systemic inflammation, blockage of blood vessels and the appearance of granulomas.The initiating factor may be an infection, such as parvovirus, or Staphylococcus aureus.
Wegener's granulomatosis can manifest itself as a variety of symptoms sharply defined, and one or two non-specific.About 90% of patients complained of typical cold and flu, making it difficult to timely diagnosis.Everyone has their own "set" of symptoms of granulomatous polyangiitis, and it takes place each in a special way.
If you have symptoms from the list, please contact your doctor as soon as possible.
Make an appointment with your doctor if you are worried about a cold, which is not treated with the usual counter drops and sprays, especially if it is accompanied by epistaxis or purulent discharge, hemoptysis, or other possible signs and symptoms of Wegener's granulomatosis.The disease can progress rapidly, so early diagnosis is crucial for the purpose of effective treatment.
Reception hours are limited, so prepare a list of questions that will help to use the time more effectively.Questions write in order of importance, if you suddenly run out of time.Here are some basic questions about the symptoms of Wegener's granulomatosis:
Most likely, your doctor will ask you many questions.Here's a list:
Examination of patients with suspected Wegener's disease includes:
treatment of Wegener's granulomatosis can be divided into two phases: remission and maintenance of remission.
main drugs in the regimen are considered cytotoxic agents:
Surgery may be required if:
treatment plan drawn up for each patient individually, according to its form of the disease, the severity of symptoms and response to therapy.
people with involvement in the process of the kidneys and severe illness often prescribe cyclophosphamide and prednisone initially.3-6 months Cytoxan changed to methotrexate or azathioprine.In the last years have proved effective biological agents (rituximab) in the treatment of Wegener's granulomatosis.
patients with mild forms of the disease prescribe a combination of methotrexate and prednisone.Gradually reduce the dose of these drugs and, finally, completely overturned, when the patient reaches the stage of remission.
All people polyangiitis with granulomatous prescribe calcium supplements to prevent osteoporosis, which can be induced by administration of hormones (prednisone).For the prevention of Pneumocystis carinii pneumonia, and to fight infections of the upper respiratory tract prescribe antibiotics such as Bactrim.
Effective treatment always involves a comprehensive approach, so each patient with Wegener's granulomatosis is assigned consulting nephrologist, ENT, ophthalmology, pulmonology, immunology and other professionals.The patient himself should be the maximum liability to carry out all prescription, monitor their status, undergo regular examinations.
Given the potential side effects of drugs used to treat granulomatous polyangiitis (for example, reduce the potential of your body to fight infections), your doctor will monitor your condition.It may also appoint agents that prevent the emergence of side effects, such as:
Cure Wegener's granulomatosis is impossible, but early diagnosis and effective treatment can help to achieve remission and return to normal life.
duration of remission is achieved and maintained by taking adequate doses of drugs, diagnostics and regular joint work of the doctor and the patient.Sometimes remission can last up to 20 years, while a person will not need to accept cytostatics and hormones.
is believed that patients with granulomatous polyangiitis risk of death in the first year of the disease in 9 times higher than in healthy people.The cause of death could be an infection, kidney failure.
probability of death was higher in patients with involvement and kidneys, and lungs, as well as those who need dialysis.
Although cyclophosphamide in patients receiving up to 90% of patients achieve remission in 50% of the disease returns.
disease discussed in the "Live Healthy»: