Comparative investigation of respiratory tract involvement in granulomatosis with polyangiitis between PR3-ANCA positive and MPO-ANCA positive cases: a retrospective cohort study

Background: The clinical characteristics of myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) positive granulomatosis with polyangiitis (GPA) remain unclear, as does the difference between MPO-ANCA positive GPA and proteinase 3 (PR3)-ANCA positive GPA, especially with regard to the details of respiratory tract involvement. We investigated the differences in clinical, radiological, and histopathological features between PR3-ANCA positive GPA and MPO-ANCA positive GPA. Methods: We retrospectively reviewed 16 patients who were newly diagnosed with GPA between December 2000 and July 2014. One patient, who was positive for both PR3-ANCA and MPO-ANCA, was excluded. Our review was based on the European Medicine Agency (EMA) algorithm. Results: Fifty-six percent of GPA patients were positive for PR3-ANCA, 38 % for MPO-ANCA, and the remaining 6 % for both. The MPO-ANCA positive group included a greater number of females (67 %). There were no statistically significant differences in laboratory data, symptoms and signs, Birmingham Vasculitis Activity Score, or CT findings between the two groups. As for upper respiratory tract involvement, the most common manifestation was paranasal sinusitis, whereas lung nodules were most common as the lower respiratory tract involvement in both groups. Although the combination therapy with prednisone and cyclophosphamide was the most common initial treatment in both groups, the relapse rate in MPO-ANCA positive cases was lower than that of PR3-ANCA positive cases (17 % and 56 %, respectively). Conclusion: A high prevalence of MPO-ANCA positive GPA was noted. No significant differences in clinico-radiological findings were observed except for the prevalence of relapse between the PR3-ANCA positive cases and MPO-ANCA positive cases, suggesting that the type of ANCA may be of little help in the diagnosis of GPA. Examination for granulomatous findings in the respiratory tract is important, even in MPO-ANCA positive cases. There is a need to accumulate more cases and conduct a further investigation in the future.