Anti-Neutrophil Cytoplasmic Antibodies: To Care or Not to Care

Anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis is a disease process with a wide range of presentations, from asymptomatic or minimally symptomatic disease with positive laboratory testing, to florid acute end-organ damage. Consensus has not been established as to the frequency and/or protocol by which ANCA testing should be repeated. We present the case of a 53-year-old woman who initially came to medical attention with persistent dyspnea and pulmonary infiltrates presumed to be due to acute exacerbation of chronic diastolic congestive heart failure. Extensive infectious disease testing was negative, but ANCA testing was positive. However, because antinuclear antibody (ANA) interference in the original sample rendered the test result difficult to interpret, the test was not repeated. The patient presented eight months after the initial hospitalization with acute hypoxemic respiratory failure requiring intubation, with an ANCA titer of 1:1280 with a negative ANA titer, and renal biopsy-proven severe crescentic glomerulonephritis. In the discussion of our case, we review the importance of interpreting ANCA testing in the correct clinical context. The ANCA laboratory testing requires cautious interpretation, and diagnosed ANCA-associated vasculitis (AAV) requires vigilance for prompt and proactive treatment.