Wegener's Granulomatosis

Wegener's granulomatosis (also known as the granulomatosis with polyangitis) is an autoimmune disease that belongs among the so-called vasculitides (inflammatory processes of blood vessels). It is mainly associated with serious impairment of the respiratory tract and kidneys.


The cause of the autoimmune inflammation is unknown. It is assumed that in some people with genetic risk, the autoimmune reaction is triggered by a banal viral infection, which causes pathological reprogramming of the immune system to target the patient's own tissues. The essence of autoimmune reaction in Wegener’s granulomatosis is particularly the existence of ANCA antibodies that are directed against intracellular content of white blood cells. Wegener's granulomatosis is typical by occurrence of granulomas that could be described as inflammatory nodes.


The most affected tissues are the respiratory tract and kidneys. Ulcerative lesions emerge in the nasal cavity; later symptoms occur such as cough, coughing up blood, chest pain and shortness of breath. Renal impairment is reflected by rapidly-progressing glomerulonephritis with presence of proteins or blood in urine. Many cases include the acute kidney failure. The patients frequently suffer from general symptoms such as weight loss, night sweats, muscle pain, joint pain and fatigue. Some cases only affect the airways and the kidneys are fine. This form of the disease has a slightly better prognosis.


Making a diagnosis is difficult. The disease can be suspected in presence of combined disability of kidneys and affection of the respiratory tract. Blood tests may show general signs of systemic inflammation including elevated sedimentation rate and CRP and impaired renal parameters. Serologic examination of the blood may confirm the presence of ANCA antibodies that are typical for advanced forms of the disease. Biopsy has an irreplaceable role to confirm the histological presence of inflammation with granulomas. The biopsy can be taken from any affected tissue, relatively often is performed the kidney biopsy.


The therapeutic protocol depends on the progress and extent of the disease. In general, we use immunosuppressive drugs to dampen the autoimmune reactions (azathioprine, cyclophosphamide, corticosteroids, etc.). The treatment is long-term and usually lasts for months. Given that the disease tends to return after ending the treatment, small doses of drugs are administered continuously. The immunosuppressive drugs unfortunately weaken the immune system and therefore, we often prefer to the biological treatment, which tries to act more accurately. However, the Wegener's granulomatosis has a poor prognosis and many people die even with maximal medical therapy.


Jiri Stefanek, MD  Author of texts: Jiri Stefanek, MD
 Contact: jiri.stefanek@seznam.cz
 Sources: basic text sources