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The most common reason to request a test for antineutrophil cytoplasmic antibodies (ANCA) is to diagnose Wegener's granulomatosis and microscopic polyangiitis and to monitor inflammatory activity in these diseases. Several retrospective and prospective studies have suggested that the demonstration of ANCA lacks sensitivity and specificity, but these series have detected ANCA with neutrophil-indirect immunofluorescence alone, have used a disease classification that did not describe microscopic polyangiitis and have included patients with inactive disease. The ‘International Consensus Statement on Testing and Reporting ANCA’ has been developed to optimize the clinical relevance of ANCA testing by the adoption of standardized testing and reporting procedures. International collaborative efforts continue to focus on improving the tests for ANCA.