Wegener's Granulomatosis

Wegener's granulomatosis is a uncommon type of inflammation of small arteries and veins (vasculitis). It classically involves inflammation of the arteries that supply blood to the tissues of the lungs, the nasal passages (sinuses), and the kidneys. "Incomplete" forms exist that only involve one of these areas. When both lungs and kidneys are affected, the condition is sometimes referred to as generalized Wegener's granulomatosis. When only the lungs are involved, the condition is sometimes referred to as limited Wegener's granulomatosis.

Wegener's granulomatosis usually affects young or middle-aged adults. Although it is uncommon in children, it can affect people at any age. The cause of Wegener's granulomatosis is not known.

Symptoms of Wegener's granulomatosis include fatigue, weight loss, fevers, shortness of breath, bloody sputum, joint pains, and sinus inflammation (sinusitis). Nasal ulcerations and even bloody nasal discharge can occur. Other areas of the body that can also become inflamed in patients with Wegener's granulomatosis include the eyes, the nerves (neuropathy), the middle ear (otitis media), and the skin resulting in skin nodules or ulcers.

Abnormal lab findings in patients with Wegener's granulomatosis include urine tests that detect protein and red blood cells in the urine (not visible to the naked eye) and x-ray tests of the chest and sinuses which detect abnormalities resulting from lung and sinus inflammation. Blood tests that detect the abnormal inflammation include the sedimentation rate (sed rate) and C-reactive protein. A more specific blood test used to diagnose and monitor Wegener's granulomatosis is the antineutrophil cytoplasmic antibody (ANCA test), which is commonly elevated when the disease is active.

The diagnosis of Wegener's granulomatosis is confirmed by detecting both abnormal cellular formations, called granulomas, and vasculitis in a biopsy of tissue involved with the inflammatory process. For examples, an open lung biopsy or a kidney biopsy are commonly used in making a diagnosis of Wegener's granulomatosis.

Source: MedicineNet.com
http://www.medicinenet.com/wegeners_granulomatosis/article.htm