Anticytoplasmic autoantibodies: their immunodiagnostic value in Wegener granulomatosis
Wegener granulomatosis is a chronic inflammatory process characterized by the development of granulomas, which are nodules or tumor-like masses, most commonly in the respiratory tract, the blood vessels and the kidneys. Antibodies directed against intracytoplasmic antigens of neutrophils, a type of white blood cell, or anticytoplasmic autoantibodies (ACPA), are present in patients with Wegener granulomatosis. It has been reported that these antibodies may be present in other disease states. The specificity of ACPA was examined in 277 patients with Wegener granulomatosis and 1,657 patients with other diseases. The ACPAs were specific for Wegener granulomatosis in approximately 99 percent of the cases. The detectability of the ACPA depended on the activity and extent of the disease. The detectability was approximately 64 percent for patients with active disease that was confined to certain regions, 36 percent for patients in full remission after initial regional symptoms, and 95 percent for patients with active generalized disease. There was a decrease in detectability to 41 percent for patients in remission after generalized disease. Therefore, the presence of ACPA in patients is specific for Wegener granulomatosis, except in a few rare cases of inflammation of the blood vessels (vasculitis). The sensitivity and levels of detectability of ACPA depend on the activity of the disease and can be used diagnostically to follow the activity of the disease. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: Annals of Internal Medicine
Association between active Wegener's granulomatosis and anticytoplasmic antibodies
Wegener's granulomatosis is a disease of unknown cause that is characterized by areas of necrosis, localized tissue death, and associated pathology of the blood vessels of the affected organs. The disease often affects lungs and kidneys. Treatment with immune blood must be started as soon as possible, and thus definitive diagnostic tests that can rapidly identify this disease are extremely important. The current study reports on a new laboratory diagnostic method that successfully identified 42 of 45 patients with active Wegener's granulomatosis, a sensitivity of 93 percent. The test was also found to be highly specific in that only two of 58 patients with a disease process similar to Wegener's tested positive, for a specificity of 97 percent. Patients with Wegener's have been found to have anticytoplasmic antibodies directed against their own white blood cells; the test makes use of this finding. The test uses blood obtained from patients suspected of having Wegener's granulomatosis and applies it to white blood cells obtained from normal donors. If the patient's blood contains the anticytoplasmic antibodies, it combines with the test cells and can be visualized by the addition of florescent dyes. Although the role of anticytoplasmic antibodies in the development of Wegener's granulomatosis remains unknown, detection of these factors can be of considerable diagnostic importance.
Publication Name: Archives of Internal Medicine
Interferon-alpha treatment of four patients with Churg-Strauss syndrome
Interferon-alpha may effectively induce remission or reduce the symptoms of Churg-Strauss syndrome. Churg-Strauss syndrome is an inflammatory blood vessel disorder marked by a significant increase in eosinophil white blood cell levels in the blood and tissues. Four patients were treated for 14-25 months with 7.5-63 million units of interferon-alpha weekly, and two patients achieved remission. The condition stabilized in the third patient, and the fourth maintained remission obtained with previous therapy.
Publication Name: Annals of Internal Medicine
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