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Wegener's granulomatosis is a disease whose first symptoms are nonspecific and indicate many diseases, which makes early diagnosis difficult. Treatment that is started too late poses a direct threat to the patient's life. What are the causes of Wegener's granulomatosis? How to recognize it? Is it possible to cure her?

Wegener's granulomatosis( granulomatosis with polyangiitis ) is a disease whose essence is necrotic granulomatous inflammation of blood vessels (small and medium ), especially in the upper and lower airways and kidneys. In the course of the disease, the immune system attacks and damages the wall of specific blood vessels, which results in inflammation leading to vasoconstriction, ischemia, infarction or organ haemorrhage.

Wegener's granulomatosis belongs to the group of systemic connective tissue diseases (rheumatic diseases). Wegener's granulomatosis occurs in about 5 in 100,000 people, with a similar frequency in men and women.

Wegener's granulomatosis: causes

The reasons for this are unknown. Some studies suggest, but do not conclude, that cANCA antibodies can cause inflammation, which can stimulate immune cells to attack.

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Wegener's granulomatosis: how to recognize the symptoms?

Initially, non-specific symptoms appear, such as fatigue, low-grade fever, loss of appetite, weight loss, muscle and bone pain. Only with the development of the disease, more characteristic symptoms appear. About 90 percent. symptoms of the respiratory system dominate:

  • chronic runny nose or very severe restriction of nasal patency, which may be accompanied by hoarseness, inflammation of the paranasal sinuses, and even otitis media
  • heavy, purulent discharge or nose bleeding, which is a sign of the formation of the so-called granulomas (ulcerative lesions) in the nose
  • as the disease develops, granulomas can pierce the septum of the nose and lead to the destruction of the cartilage of the nasal bridge and its "collapse" (the so-called saddle nose)

When the granulomas occupy the upper and lower airways, there is shortness of breath and coughingand chest pain and sometimes haemoptysis. Changes in the kidneys appear quite late, moreover, they are often completely asymptomatic and only a careful examination of the urine allows them to be visualized. The inflammatory process may also include the nervous system, eyes, ears, skin, muscles and joints, the digestive tract, and in rare cases also the heart, giving symptoms such as:

  • eyes - in 40 percent patients have eye changes, such as inflammation of the sclera, episcleritis, conjunctivitis of the uveal membrane or the tear duct, less often - exophthalmos
  • skin - rash, palpable lumps under the skin, bleeding under the skin
  • musculoskeletal system - recurrent arthritis
  • digestive system - abdominal pain, diarrhea (may be bloody)
  • nervous system - sensory disturbances, even a stroke
  • oral cavity - recurrent gingivitis, often painless mucosal ulcerations

The course of the disease varies from patient to patient and depends on the type and size of the vessels involved and the activity of the inflammatory process. In some it is mild and slow, in others it can progress very quickly and lead to a life-threatening condition.

Important

Recurrent nosebleeds, bloody diarrhea, hearing loss, painless mouth ulcers, skin lesions, hemoptysis or haematuria are symptoms that occur in the course of several dozen or even hundreds of diseases. Therefore, they do not always have to indicate granulomatosis with polyangiitis, especially since it is a rarely diagnosed disease. However, these are alarming symptoms, which means that if they occur, see a doctor as soon as possible.

Wegener's granulomatosis: diagnosis

To identify granulomatosis with polyangiitis, a blood test is usually performed to determine inflammatory markers (ESR or CRP) and the presence of antibodies (cANCA + anti-neutrophil cytoplasmic antibodies). Urine tests are also performed (the presence of protein in the urine suggests kidney damage) and an X-ray or CT scan of the chest to visualize any changes in the respiratory tract.

Wegener's Granulomatosis: Treatment

Wegener's granulomatosis is an incurable disease. The activities of doctors focus mainly on bringing her into remission. For this purpose, the most commonly used drugs from the group of glucocorticosteroids and cyclophosphamide - agents that weaken the excessively strong immune system of the patient.

A specific marker of this disease is the presence of c-ANCA antibodies.

In severe cases of disease - whenstandard treatment does not bring results, and the patient struggles with severe kidney damage and high levels of cANCA antibodies - plasmapheresis can be used. It is a method of cleaning (filtering out) some particles from the blood, including cANCA antibodies. Unfortunately, the procedure is expensive and only has a temporary effect. As time goes on, the levels of antibodies build up and the disease returns. Dialysis is required if the kidneys become severely damaged and fail, and surgery is required in those who have narrowed the larynx due to scarring, or a haemorrhage in the lungs or digestive tract.

Wegener's granulomatosis: prognosis

The prognosis depends on when the disease is diagnosed, the organs involved and the treatment methods. Untreated, it is given about half a year of life from the moment of diagnosis. Among patients treated in 87 percent. patients were observed to survive from 6 months to 24 years.

About the authorMonika Majewska A journalist specializing in he alth issues, especially in the areas of medicine, he alth protection and he althy eating. Author of news, guides, interviews with experts and reports. Participant of the largest Polish National Medical Conference "Polish woman in Europe", organized by the "Journalists for He alth" Association, as well as specialist workshops and seminars for journalists organized by the Association.

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Immune system