Abstract
BackgroundMyeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) is often positive in patients with interstitial lung disease (ILD), which is also often present in patients with microscopic polyangiitis (MPA). A possible association between MPO-ANCA, MPA, and idiopathic ILD remains unclear. The objective of this study was to determine whether high-resolution computed tomography (HRCT) classification based on recent idiopathic pulmonary fibrosis guideline and specific CT findings can obtain new knowledge of prognostic factors in all MPO-ANCA-positive patients with ILD including both idiopathic ILD and MPA-ILD.MethodsWe analyzed 101 consecutive MPO-ANCA-positive patients with respiratory disease. We assessed the diagnostic accuracy of CT findings, HRCT pattern, and specific radiological signs. Prognostic predictors were determined using Cox regression models.ResultsSubjects with chronic ILD included 22 patients with MPA-ILD and 39 patients with ILD but without MPA. A quarter of the patients were radiological indeterminate for usual interstitial pneumonia (UIP) pattern, which resulted in a better prognosis than that for UIP pattern. “Increased attenuation around honeycomb and traction bronchiectasis” and “anterior upper lobe honeycomb-like lesion” were found to be highly frequent radiological findings (39% and 30%, respectively). In addition, the latter finding was a significant negative prognostic factor.ConclusionsRadiological indeterminate for UIP was a useful HRCT classification in MPO-ANCA-positive patients with ILD. In addition, anterior upper lobe honeycomb-like lesion was found to be specific radiological finding that was a significant prognostic factor. The present results might aid in the assessment of appropriate strategies of diagnosis in these patients.
Highlights
Antineutrophil cytoplasmic antibody (ANCA) is well known to be pathogenic and to have diagnostic value for ANCA-associated vasculitis, and myeloperoxidaseANCA (MPO-ANCA) has been thought to be related to the pathogenesis of microscopic polyangiitis (MPA) [1]
MPO-ANCA positivity is often found in patients with interstitial lung disease (ILD) in clinical practice, a possible association between MPO-ANCA, MPA, and idiopathic interstitial pneumonias (IIPs) remains unclear
Some recent studies showed that MPOANCA positivity was associated with subsequent MPA development in patients initially diagnosed as having IIPs, especially usual interstitial pneumonia/idiopathic pulmonary fibrosis (UIP/Idiopathic pulmonary fibrosis (IPF)) [2,3,4]
Summary
Antineutrophil cytoplasmic antibody (ANCA) is well known to be pathogenic and to have diagnostic value for ANCA-associated vasculitis, and myeloperoxidaseANCA (MPO-ANCA) has been thought to be related to the pathogenesis of microscopic polyangiitis (MPA) [1]. Most reports mentioned that UIP pattern was the main pattern on high-resolution computed tomography (HRCT), an overlapping or indeterminate pattern (i.e., unclassifiable pattern other than UIP, non-specific interstitial pneumonia, and organizing pneumonia) may present to some extent in the MPO-ANCA-positive population with ILD because pathologically, MPO-ANCApositive patients with ILD showed more prominent inflammatory cell infiltration and cellular bronchiolitis [2, 6] To clarify these issues, we aimed to investigate prognostic factors of mortality in MPO-ANCA-positive patients with ILD by using HRCT pattern classification based on the recent IPF guideline [7]. The objective of this study was to determine whether high-resolution computed tomography (HRCT) classification based on recent idiopathic pulmonary fibrosis guideline and specific CT findings can obtain new knowledge of prognostic factors in all MPO-ANCA-positive patients with ILD including both idiopathic ILD and MPA-ILD
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
