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. 2015 Jul 30:15:78.
doi: 10.1186/s12890-015-0068-1.

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

Satoshi Ikeda  1 , Machiko Arita  2 , Kenta Misaki  3 , Yumiko Kashiwagi  4 , Yuhei Ito  5 , Hirotaka Yamada  6 , Machiko Hotta  7 , Akihiro Nishiyama  8 , Akihiro Ito  9 , Maki Noyama  10 , Takashi Koyama  11 , Kenji Notohara  12 , Tadashi Ishida  13
Affiliations

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

Satoshi Ikeda et al. BMC Pulm Med. .

Abstract

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.

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Figures

Fig. 1
Fig. 1
Classification of the patients according to the European Medicine Agency algorithm. According to the EMA algorithm, all PR3-ANCA positive cases, three of six MPO-ANCA positive cases, and one double-positive case met the ACR criteria. The remaining three MPO-ANCA positive cases were diagnosed as GPA on the basis of the surrogate markers and positivity for ANCA without histological proof of granuloma or necrotizing vasculitis. Abbreviations: EMA, European Medicine Agency; ACR, American College of Rheumatology; CHCC, Chapel Hill Consensus Conference
Fig. 2
Fig. 2
Representative photographs of computed tomography (CT) scans. a Initial CT scan of 63 y.o. male positive for PR3-ANCA shows a solitary nodule and wall thickning of lobar bronchi. b Initial high-resolution CT scan of 66 y.o female positive for MPO-ANCA shows several nodules, ground glass opacity, wall thickning of lobar and segmental bronchi, and pleural effusion
See this image and copyright information in PMC

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