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Review
. 2025 Jun 26;45(7):159.
doi: 10.1007/s00296-025-05896-2.

Pulmonary arteritis in the course of granulomatosis with polyangiitis

Aleksandra Sobuń  1 , Marta Madej  2 , Ewa Mroczek  3 , Maciej Guziński  4 , Piotr Wiland  2 , Agata Sebastian  2
Affiliations
Review

Pulmonary arteritis in the course of granulomatosis with polyangiitis

Aleksandra Sobuń et al. Rheumatol Int. .

Erratum in

Abstract

Granulomatosis with polyangiitis (GPA) is a rare disease that belongs to the group of necrotizing systemic vasculitis, which is characterized by the involvement of small and medium-sized blood vessels, the formation of granulomas, and the presence of proteinase 3-anti-neutrophil cytoplasmic antibodies (PR3-ANCA). The disease typically involves the upper and lower respiratory tract and kidneys, but other organs and systems may also be involved. A rare manifestation of the disease is the involvement of large vessels, which may occur in the form of aneurysms, dissection and stenosis. We present the case of a 39-year-old male patient who was diagnosed with GPA several years ago, with the involvement of lungs, kidneys, skin and inflammation of the ascending aorta. The patient currently reported fever, cough and dyspnea. The imaging studies showed stenosis of the right and left pulmonary arteries. The treatment included glucocorticosteroids and rituximab. A review of the literature on pulmonary artery involvement in granulomatosis with polyangiitis was performed, seven case reports meeting such criteria were found.

Keywords: ANCA-associated vasculitis; Granulomatosis with polyangiitis; Large vessel vasculitis; PET/CT imaging in vasculitis; Pulmonary arteries; Rituximab.

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Conflict of interest statement

Declarations. Conflict of interest: No part of this manuscript, including the text or graphics, are published elsewhere in whole or in part. All authors have no conflict of interest related to this article. All authors take full responsibility for the integrity and accuracy of all aspects of the work. The data that support the findings of this study are available on request from the corresponding author. This work was not supported by any funding. Informed consent: Informed consent was obtained from the patient involved in the study.

Figures

Fig. 1
Fig. 1
White arrow shows a narrowing of the right pulmonary artery wall in angio-CT scan from 2019
Fig. 2
Fig. 2
On follow-up examination in 2023, progression of narrowing in the right pulmonary artery is showed
See this image and copyright information in PMC

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