PubMed · 12686682
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Tiberiu Hershcovici, Joseph Shohat, Vered Schechner, Ana Tobar, Yitzhak Beigel. 2003. Head or tail?. https://doi.org/10.1093/ndt%2Fgfg052
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PURPOSE OF REVIEW: Antineutrophil cytoplasmic antibodies are closely associated with Wegener granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome and have contributed to new pathogenetic concepts and improved nomenclature of systemic vasculitides (antineutrophil cytoplasmic antibody-associated vasculitides). However, the application of antineutrophil cytoplasmic antibody testing as a clinical diagnostic tool is still regarded as controversial. This review summarizes the most recent developments in the field, identifies areas of uncertainty, and gives practical guidelines. RECENT FINDINGS: The problems of antineutrophil cytoplasmic antibody testing include the diversity of antineutrophil cytoplasmic antibody target antigens, assay standardization and performance, the application of antineutrophil cytoplasmic antibody testing in a clinical setting with a low pretest probability, and, finally, the widespread assumption that antineutrophil cytoplasmic antibody titers alone may closely reflect disease activity and therefore may be used to guide therapy. SUMMARY: Recent findings demonstrate that the combined use of indirect immunofluorescence tests and solid phase assays to detect antineutrophil cytoplasmic antibody directed against myeloperoxidase and proteinase 3 can minimize the occurrence of false-positive antineutrophil cytoplasmic antibody results. Furthermore, the yield of antineutrophil cytoplasmic antibody testing can be improved by the use of a well-standardized test, adherence to published guidelines, and restricting the use of the tests to clinical situations with a rather high pretest probability for antineutrophil cytoplasmic antibody-associated vasculitides. However, treatment decisions should be based on the clinical presentation of the patient and histologic findings and not on the results of antineutrophil cytoplasmic antibody testing alone.
Standard therapy of Wegener's granulomatosis is prednisolone in combination with cyclophosphamide. Cyclophosphamide is continued for 15-18 months after diagnosis. During therapy 26% of the patients have severe or life-threatening side effects, mostly infections. Furthermore, there are multiple long-term side effects such as an increased incidence of malignancies, especially bladder carcinoma. During the last 10 years the European vasculitis study group (www.vasculitis.org) organized several randomized clinical trials to determine optimal treatment. Recently, it was demonstrated in one of these trials that in patients with generalized ANCA-associated vasculitis (vasculitis associated with antineutrophil cytoplasmic autoantibodies) withdrawal of cyclophosphamide and substitution by azathioprine after remission did not increase the rate of relapse and was safe. Wegner's granulomatosis is not a fatal disease anymore. Sometimes, however, patients lose their job. Fortunately, this occurs only in a small percentage of patients.
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