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Wegener's Granulomatosis

Medical Author: William C. Shiel Jr., MD, FACP, FACR

What is 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.

What are symptoms of Wegener's granulomatosis?

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.

How is Wegener's granulomatosis diagnosed?

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.



Next: How is Wegener's granulomatosis treated? »

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Wegener's Granulomatosis

What is vasculitis?

Vasculitis is a general term for a group of uncommon diseases that feature inflammation of the blood vessels. The blood vessels of the body are referred to as the vascular system. The blood vessels are comprised of arteries that pass oxygen-rich blood to the tissues of the body and veins that return oxygen-depleted blood from the tissues to the lungs for oxygen. Vasculitis is characterized by inflammation in and damage to the walls of various blood vessels.

Each of the vasculitis diseases is defined by certain patterns of distribution of blood vessel involvement, particular organ involvement, and laboratory test abnormalities. As a group, these diseases are referred to as vasculitides.

The word vasculitis is derived from the Latin "vasculum", vessel + "- itis", inflammation. Another term for vasculitis is angiitis. When arteries are the inflamed blood vessels, the condition is also referred to as arteritis. When the veins are ...

Read the Vasculitis article »











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