ANCA and biopsy. The most useful blood test is an anca with titer and pattern, and pr3/mpo confirmation. Biopsy of the affected tissue is also important. Imaging studies of the lungs and sinuses can be useful as well as blood and urine tests looking for kidney damage. Finally, EMG might be needed if there is concern for nerve damage.
Clinical evaluation. Clinical evaluation with x-rays and biopsy.
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.
WG. Wegener’s granulomatosis (WG) is a rare disease that causes the walls of blood vessels to become inflamed (called vasculitis). This limits blood flow to tissues and can affect any organ. WG is a type of autoimmune disease. This means the body’s immune system attacks its own tissues. Its cause is unknown.
Yes. Wegener's granulomatosis with polyangiitis is an incurable form of vasculitis (inflammation of blood vessels) that affects the lungs, kidneys, and other organs. Due to its 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 treatment. Since that is an average figure, some will live longer.