Wegener’s granulomatosis (WG) is a rare blood vessel disease that can cause symptoms in the nasal sinuses, lungs and kidneys as well as other organs. This is a complex and potentially serious disease. However, with prompt diagnosis, WG can be treated effectively.
WG belongs to a group of diseases characterized by the presence of blood vessel inflammation (vasculitis). In WG, organ damage occurs as a result of vasculitis involving the small‐ and medium‐sized blood vessels, and from a type of tissue injury called granulomatous inflammation. [A granuloma is a mass or nodule of inflamed tissue, usually seen on biopsies of affected organs.]
Most commonly, WG affects the nasal sinuses, lungs and kidneys, but can also affect the eyes, ears, skin, nerves, joints and other organs. Because of the variety of potential organ involvement, a wide range of symptoms can develop over days to months. For 90% of people, the first symptoms appear in the respiratory tract (e.g., nose and lungs) and include nasal congestion, frequent nosebleeds, shortness of breath, and cough that may produce bloody phlegm. Other early symptoms can include joint pain, decreased hearing, skin rashes, eye redness and/or vision changes, fatigue, fever, appetite and weight loss, night sweats, and numbness or loss of movement in the fingers, toes or limbs.
Well‐studied therapies have proven effective in treating WG. Although permanent organ damage can still occur, the medications used to treat WG can improve or even resolve organ injury in many instances.
The cause of WG is unknown.
This rare disease is estimated to affect 3 out of every 100,000 people, and men and women equally. The condition can occur at any age, but most often between the ages of 40 and 50.
The impact of WG varies greatly between individuals. It’s influenced by the severity of their illness, the organs involved, and any complications related to the disease or its treatment. People with WG need ongoing checkups that include laboratory studies, imaging tests, and clinic visits with their doctor. Such monitoring is essential to follow the course of WG and to detect or prevent short‐ or long‐term treatment‐related complications.
Even with effective treatment, recurrences (relapses) are common. Relapses may resemble or differ from the initial onset so new symptoms should be reported to the medical practitioner as soon as possible. Regular doctor visits together with monitoring of laboratory tests and imaging studies can help to detect relapses early.