Wegener's Granulomatosis

Wegener's granulomatosis is vasculitis that often begins with inflammation of the lining of the nose, sinuses, throat, or lungs and may progress to inflammation of blood vessels throughout the body (generalized vasculitis) with potentially fatal kidney failure.

Wegener's granulomatosis can occur at any age and is twice as common in men as in women. Its cause is unknown. It resembles an infection, but no infecting organism has been found. Wegener's granulomatosis is thought to be caused by an allergic response to a trigger that has not yet been identified. The result is a potent, inappropriate immune reaction that damages many tissues in the body.

The disorder produces inflammation of blood vessels (vasculitis) and an unusual type of inflammatory tissue called a granuloma, which ultimately destroys normal tissue.

Symptoms

The disorder may begin suddenly or gradually. The first symptoms usually affect the upper respiratory tract--the nose, sinuses, ears, and windpipe (trachea)--and may include severe nosebleeds, sinusitis, middle ear infections (otitis media), coughing, coughing up of blood, and shortness of breath. The lining of the nose may become red and rough, bleeding easily. A fever, a generally sick feeling (malaise), loss of appetite, joint aches and swelling, and inflammation of the eye or ear may develop. The disease may affect arteries to the heart, causing chest pain or a heart attack, or it may affect the brain or spinal cord, producing symptoms that resemble those of several neurologic diseases.

The disorder may progress to a generalized (disseminated) phase, with inflammation of blood vessels throughout the body. As a result, sores appear on the skin and spread extensively; they can severely scar the skin. Kidney damage, common at this stage of Wegener's granulomatosis, ranges from mild impairment to life-threatening kidney failure. Severe kidney disease causes high blood pressure and symptoms resulting from the buildup of wastes in the blood (uremia). Anemia is common and can be severe.

In some cases, only the nasal passages, sinuses, and upper airways are affected for many years; this less severe disorder is called limited Wegener's granulomatosis. However, progression to the more serious disorder is possible.

Diagnosis

Wegener's granulomatosis must be diagnosed and treated early to prevent complications, including kidney disease, lung disease, heart attacks, and brain damage.

A doctor usually recognizes the distinctive pattern of symptoms. Although blood test results cannot specifically identify Wegener's granulomatosis, they can strongly support the diagnosis. A blood test can detect antineutrophil cytoplasmic antibodies in the blood, which strongly suggest this disease. If the nose, throat, or skin is not affected, diagnosis can be difficult because the symptoms and x-rays can resemble those of several lung diseases.

A chest x-ray may show cavities or dense areas in the lungs that look like cancer. Doctors can make a definite diagnosis only by examining a small piece of tissue under a microscope (a biopsy); the tissue sample may be taken from an affected area, such as the nasal passages, airways, or lungs. Skin and kidney biopsies may be less helpful.

Treatment

Corticosteroids may be used alone to treat the early symptoms of Wegener's granulomatosis. However, most people also need another immunosuppressive drug, such as cyclophosphamide.

The generalized form of Wegener's granulomatosis is treated with immunosuppressive drugs, such as cyclophosphamide and azathioprine, which control the disease by reducing the body's inappropriate immune reaction. Such use significantly improves the prognosis because, without treatment, this form of the disorder is fatal. Treatment is usually continued for at least a year after the symptoms disappear. Corticosteroids, given at the same time to suppress inflammation, can usually be tapered off and discontinued.

For people who are receiving immunosuppressive drugs, a doctor treats any suspected infection as early as possible because of the body's decreased ability to fight infections. Pneumonia is particularly common when the lungs are damaged. An antibiotic may be used to prevent infections in people who have been taking immunosuppressive drugs for years.