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Diagnostic Tests for Wegener's granulomatosis

Wegener's granulomatosis: Diagnostic Tests

The list of diagnostic tests mentioned in various sources as used in the diagnosis of Wegener's granulomatosis includes:

Home Diagnostic Testing

These home medical tests may be relevant to Wegener's granulomatosis:

Tests and diagnosis discussion for Wegener's granulomatosis:

To treat people with Wegener’s granulomatosis most effectively, doctors must diagnose the disease early in its course. There are no blood tests that a doctor can use to diagnose Wegener’s granulomatosis, but blood tests are important to rule out other causes of illness and to determine which organ sites may be affected. Most blood tests are nonspecific and can only suggest that a person has an inflammatory process. Anemia (low red blood cell count), elevated white blood cell count and platelet count, and an elevated sedimentation rate are commonly found in people with Wegener’s granulomatosis. If the kidneys are involved, red blood cells and structures called red blood cell casts are visible in the urine when viewed under a microscope, and the blood tests measuring kidney function (creatinine and BUN) may show abnormalities.

X-ray results can be very helpful in diagnosing Wegener’s granulomatosis. People with lung involvement will have abnormal chest x-rays, which may show one or many fluffy infiltrates, solid nodules, or cavities. Sinus x-rays or computed tomography (CT) scans in people with sinus involvement may show thickening of the sinus lining.

Many patients with active Wegener's granulomatosis have a blood test that reveals the presence of a specific type of antibody called antineutrophil cytoplasmic antibodies (ANCA) (an antibody is a disease-fighting protein). Although a positive ANCA test is useful in supporting a suspected diagnosis of Wegener’s granulomatosis, in most instances it is not used by itself to make a diagnosis of this disorder. The ANCA test may be negative in some patients with active Wegener’s granulomatosis.

Currently, the only definite way to diagnose Wegener’s granulomatosis is by performing a biopsy of an involved organ site (usually the sinuses, lung, or kidney). The tissue is examined under the microscope to confirm the presence of vasculitis and granulomas (a specific type of inflammation), which together are diagnostic features of the disease. A biopsy is very important both to confirm the presence of Wegener’s granulomatosis and also to assure the absence of other disorders that may have similar signs and symptoms.

Treatment

With the appropriate treatment, the outlook is good for patients with Wegener’s granulomatosis. In a study of 158 patients who were treated at the National Institutes of Health (NIH), 91 percent of them markedly improved. After 6 months to 24 years of follow-up, 80 percent of the patients survived.

In most cases, standard therapy consists of a combination of a glucocorticoid drug that reduces inflammation and a cytotoxic drug that interferes with the abnormal growth of cells.

Prednisone is the most common glucocorticoid drug (a steroid) that is used. Prednisone is similar to hydrocortisone, the natural glucocorticoid hormone produced by the body. It is chemically different from the anabolic steroids that have been used by athletes and is given in doses much higher than the body normally produces. Prednisone is usually administered as a single morning dose in an attempt to imitate how the body normally secretes hydrocortisone. When the person’s illness improves, the prednisone dose is gradually decreased and converted to an every other day dosing schedule, usually over a period of 3 to 4 months. With further improvement in the disease, the prednisone is very gradually decreased and discontinued completely after approximately 6 to 12 months. When prednisone is taken by mouth, the body stops making its own natural hydrocortisone. As the prednisone dose is gradually reduced the body will resume making hydrocortisone again. It is extremely important that prednisone never be stopped suddenly because the body requires prednisone (or hydrocortisone) for its function and may not be able to immediately make what it needs.

Cyclophosphamide (CytoxanÔ) is the most commonly used cytotoxic drug. Cyclophosphamide is taken once a day by mouth. It is important for a patient to take the drug all at once in the morning followed by drinking a large amount of fluid. Although the initial dose of cyclophosphamide is based on the patient’s weight and kidney function, the doctor may adjust the dosage based on the blood counts, which are monitored closely to be sure that the white blood cell count is maintained at a safe level. Cyclophosphamide is continued for a full year beyond that point at which the disease has become quiet (is in remission). The dose of cyclophosphamide is then decreased gradually and eventually discontinued.

Cyclophosphamide and prednisone are both powerful drugs that suppress the immune system. Although these medications are beneficial in treating Wegener’s granulomatosis, patients and their doctors should be aware that the drugs potentially have serious side effects. Careful monitoring by the doctor is very important. Because these drugs suppress the immune system, they can affect the body’s ability to fight off infection. Patients should report immediately any symptoms of infection and, specifically, any fever to their doctors. Prednisone can cause weight gain, cataracts, brittle bones, diabetes, and alterations in mood and personality. Cyclophosphamide can cause bone marrow suppression (lowering of blood counts), sterility, hemorrhagic cystitis (bleeding from the bladder) as well as other serious side effects.

Approximately half of people with Wegener’s granulomatosis may experience a return (relapse) of their disease. This occurs most frequently within two years of stopping medication, but potentially can occur at any point both during treatment or after stopping treatment. Thus, it is extremely important that patients continue to see their physicians regularly, both while they are on these medications, as well as after the medications have been stopped. Even while on medication, many patients are able to lead relatively normal lives and will remain in remission after therapy has been stopped completely.

Research

Since the 1970s, research physicians at the National Institute of Allergy and Infectious Diseases (NIAID), a component of NIH, have been interested in Wegener’s granulomatosis. NIAID scientists first introduced the combination of a glucocorticoid with cyclophosphamide for treating people with this disease. This dramatic breakthrough remains the standard of treatment. Despite this, researchers realize that these medicines have serious side effects and cannot be tolerated by all people. Therefore, NIH researchers continue to study Wegener’s granulomatosis to understand the causes of the disease and to develop new treatments. NIAID is conducting several studies to investigate new treatment regimens. These studies each have individual entry criteria but are open to patients who have a definitive diagnosis of Wegener’s granulomatosis and who have active disease.

Further Information

For information about enrolling in an NIAID clinical study at NIH's facility in Bethesda, Maryland, the patient's personal physician should call or write to:

Dr. Michael C. Sneller
Head, Immunologic Diseases Section
or
Dr. Carol A. Langford
Immunologic Diseases Section
Laboratory of Immunoregulation
National Institute of Allergy and Infectious Diseases
National Institutes of Health
Building 10, Room 11B-13, MSC 1876
10 Center Drive
Bethesda, MD 20892-1876
301/496-1124

For more information on patient support groups, contact:

Wegener's Foundation, Inc.
Attention: Ms. Linda Baltrusch
3705 South George Mason Drive
Suite 1813 South
Falls Church, VA 22041
703/931-5852

Wegener's Granolomatosis Support Group, Inc. (International)
P.O. Box 28660
Kansas City, MO 64188-8660
800/277-WGSG (9474)
http://www.wgsg.org/

(Source: excerpt from Wegener's Granulomatosis, NIAID Fact Sheet: NIAID)
 

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