Wegener's granulomatosis

Alternative names:

midline granulomatosis

Definition:

A rare disorder associated with chronic tissue inflammation due to inflammation of blood vessels (vasculitis), and granulomas (aggregation of cells) in the nasal passages, lungs, and kidneys.

Causes, incidence, and risk factors:

The cause is unknown, but it is thought to be due to an autoimmune disorder and is often classified as one of the rheumatic diseases. Wegener's is characterized by destructive lesions that develop in the upper and lower respiratory tract and the kidney. In the kidney these lesions cause a glomerulonephritis that may result in kidney failure. It occurs most often between the ages of 30 to 50. It is rare in children, but has been seen in infants as young as 3 months old.

Early symptoms are often systemic and may include fatigue, malaise (an ill feeling), fever, and a sense of discomfort around the nose and sinuses. Persistent fever without obvious cause (fever of undetermined origin - FUO) may be an initial finding. Night sweats may accompany the fever. Anorexia and weight loss are common. Significant symptoms often appear as pulmonary manifestations: cough, bloody sputum, and progressive breathing difficulties (later requiring oxygen). Involvement of the sinuses and nose may produce a bloody discharge, pain, and ulcers and sores around the opening of the nose. Skin lesions are common, but there is no one characteristic lesion associated with the disease. Kidney disease may have no symptoms but is readily 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.