Wegener's granulomatosis is a very rare disease that affects many different organs and systems of the body. It mainly attacks the respiratory system (sinuses, nose, windpipe, and the lungs) and the kidneys. One of the main features of the disease is an inflammation of the blood vessels (vasculitis). The inflammation narrows the blood vessels and reduces the blood flow to the affected organs. This destroys tissues and damages vital organs.
Wegener's granulomatosis (WG) is not a contagious disease, and there is no evidence to suggest that it is hereditary either. It is a very rare disease, affecting only 1 in every 30,000-50,000 people. About 500 new cases are diagnosed each year. The disease can occur at any age, however, it mostly affects individuals in their 30s and 40s. It affects males and females equally. Ninety seven percent of all patients are Caucasian, 2% are Black and 1% are of another race.
Causes and symptoms
No viral, bacterial, or other causative agent has yet been identified for WG. It is thought to be an autoimmune disease, meaning that the body's immune system attacks "itself," that is, the body's own tissues.
Whenever there is an infection in the body, proteins called antibodies, which are capable of attacking the infectious agent, are formed in the blood. In WG, the antibodies that are formed are directed against the white blood cells of the immune system. They are therefore called "auto-antibodies" (antibodies against one's own body cells). These auto-antibodies bind to the blood cells and forms clumps known as immune complexes. The complexes accumulate in the tissues and the blood vessels, leading to a tumor-like (granulomatous) inflammation of the blood vessels. This slows down the blood flow to the different organs and tissues, causing damage and resulting in the many symptoms of WG.
The symptoms of WG, and the severity of the symptoms, vary from patient to patient. One of the most common features is a chronic runny nose and other cold-like symptoms that do not respond to standard treatment. The cold symptoms gradually worsen and could lead to sinusitis (inflammation of the sinuses), middle ear infection (otitis media), cough, coughing of blood, and inflammation of the lung (pleuritis and pneumonia). Other symptoms include fever, fatigue, loss of appetite, weight loss, joint pain, night sweats, change in urine color, and weakness.
Kidney (renal) disease is the most serious development of WG. Patients who do not have renal disease are said to have "Limited Wegener's."
Lata Cherath PhD, The Gale Group Inc., Gale, Detroit,