Systemic vasculitis. Wegener's is a systemic vasculitis associated with the presence of specific blood tests known as a canca or pr3 antibodies. It usually presents as chronic sinusitis and more rarely pneumonia but can effect other areas of the body especially the kidneys, eyes, heart and nervous system. It is very serious but newer medications have provided effective treatment in most cases.
Inflammation.... ..Caused perhaps by an auto-immune response that can damage the lungs, sinuses, kidneys and occasionally small nerves. Its actually the blood vessels specifically in those organs I mentioned that get inflamed. Treatment is getting better, and more well tolerated as we understand the best, and safest, combinaiton (s) and sequences of drugs to use. The cause is not known.
Autoimmune disease. Like all autoimmune diseases, the cause is not really known, but likely a combination of genetic factors and environmental exposures (e.g., infections, chemicals, pollutants). Treatment depends on how severe disease it is. Typically steroids (e.g. Prednisone) and if severe, Cytoxan or rituxan (rituximab). If limited disease (just nasal/upper airway involvement), Methotrexate may be sufficient.
Disease or Syndrome? Wegener's granulomatosis (wg), more recently granulomatosis with polyangiitis (wegener's) (gpa), is an incurable form of vasculitis (inflammation of blood vessels) that affects the nose, lungs, kidneys, and other organs. Due to its end-organ damage, it is life-threatening and requires long-term immunosuppression. five-year survival is up to 87%, with some of the mortality due to toxicity of rx.
Serious illness. Wegner's granulomatous angiitis is caused by antibodies against a particular protein in neutrophils. Once uniformly fatal, it is now usually managed with strong medications by specialists. The eyes, ears, throat, lungs and kidneys are the usual targets. Wegener's greatness as a physician is tarnished by his having served the third reich, though not in the death camps; some avoid his name.
See details. This is a serious systemic vasculitis characterized by inflammatory in medium-sized arteries. Clinical manifestation often start with chronic sinusitis or recurrent lung infiltrates but multiple organs can be involved including the kidneys. It must be treated aggressively to prevent serious complications. See a rheumatologist.