Clinical implications of ANCA positivity in a hospital setting: a tertiary center experience

ANCA testing plays an established critical role in the diagnosis of ANCA Associated vasculitis (AAV). The spectrum of diseases associated with positive ANCA has recently broadened, thus calling into question the diagnostic implications of ANCA positivity in a hospital setting. We retrospectively studied all adult patients who had a positive ANCA test (by Indirect Immunofluorescence (IIF), ELISA or both) performed over the span of 19 years. Subjects were then divided into discordant (positive on one assay) and concordant ANCA (positive on both assays) groups based on their ANCA positivity status. The two groups were then compared with regards to their demographic, clinical and laboratory characteristics, the indication for ANCA testing in both groups and their final diagnoses. Of the 9189 ANCA tests ordered during the 19-year span of the study, 389 (4.2%) were positive. Two hundred and forty subjects met the exclusion criteria (patients aged less than 18 years or the lack of clinical and laboratory data in the medical file) thus resulting in a final cohort of 149 subjects. Of them, 122 subjects had discrepant ANCA results and 27 had matching ANCA results. Most cases in the discrepancy group were IIF positive and ELISA negative (86.8%). The diagnosis of AAV was highly unlikely in cases with discrepant IIF and ELISA serologies compared to cases with matching IIF and ELISA serologies (4.1% versus 44.4%, p value < 0.001). The diagnosis of AAV in unlikely in subjects with discrepancies between IIF and ELISA, particularly with only positive IIF.