Midline granulomatosis
Key points
- Wegener's Granulomatosis is a rare disorder which causes inflammation of blood vessels (vasculitis) in the upper respiratory tract (nose, sinuses, ears), lungs, and kidneys.
- The cause is unknown, but Wegener's Granulomatosis is thought to be an autoimmune disorder and is often classified as one of the rheumatic diseases.
- Early symptoms may include fatigue, malaise (an ill feeling), fever, and a sense of discomfort around the nose and sinuses.
- Treatment Treatment with corticosteroids, cyclophosphamide, methotrexate, or azathioprine produces long-term remission in over 90% of affected people.
Alternative names
Wegener's granulomatosis
Definition
Wegener's Granulomatosis is a rare disorder which causes inflammation of blood vessels (vasculitis) in the upper respiratory tract (nose, sinuses, ears), lungs, and kidneys. Many other areas of the body may also be affected, with arthritis (joint inflammation) occurring in almost half of all cases. The eyes and skin may also be affected.
Causes, incidence, and risk factors
The cause is unknown, but Wegener's Granulomatosis is thought to be an autoimmune disorder and is often classified as one of the rheumatic diseases. Destructive lesions develop in the upper and lower respiratory tract and the kidney. In the kidney, these lesions cause glomerulonephritis that may result in hematuria (blood in the urine) and kidney failure.
It occurs most often between the ages of 30 and 50, and men are affected twice as often as women. It is rare in children, but has been seen in infants as young as 3 months old.
The kidney disease can progress rapidly, with kidney failure occurring within months of the initial diagnosis. If untreated, kidney failure and death occur in more than 90% of all patients with Wegener's granulomatosis.
Symptoms
Early symptoms may include fatigue, malaise (an ill feeling), fever, and a sense of discomfort around the nose and sinuses. Upper respiratory infections such as sinusitis or ear infections frequently precede the diagnosis of Wegener's Granulomatosis. Other upper respiratory symptoms include nose bleeds, pain, and sores around the opening of the nose.
Persistent fever without an obvious cause (fever of undetermined origin - FUO) may be an initial symptom. Night sweats may accompany the fever. Loss of appetite and weight loss are common. Skin lesions are common, but there is no one characteristic lesion associated with the disease.
Kidney disease is necessary to make the definitive diagnosis of Wegener's Granulomatosis. The urine may be bloody, which often first appears as red or smoky urine. There may be no symptoms, but is easily diagnosed with laboratory studies.
Eye problems develop in a significant number of patients and may range from a mild conjunctivitis to severe inflammation of the eyeball and the tissues around the eyeball. Additional symptoms include:
- weakness
- loss of appetite
- weight loss
- bloody discharge from the nose
- pain over the sinuses
- sinusitis
Written and revised by
Andrey Nailor, M.D.
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