Cho U, Kim SK, Ko JM, Yoo J. Unusual presentation of granulomatosis with polyangiitis causing periaortitis and consequent subclavian steal syndrome: A case report. World J Clin Cases 2021; 9(6): 1433-1438 [PMID: 33644212 DOI: 10.12998/wjcc.v9.i6.1433]
Corresponding Author of This Article
Jinyoung Yoo, MD, PhD, Doctor, Professor, Department of Hospital Pathology, St. Vincent’s Hospital, College of Medicine, The Catholic University of Korea, Ji-dong 93, Paldal-gu, Seoul 06591, South Korea. jinyyoo@catholic.ac.kr
Research Domain of This Article
Rheumatology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Clin Cases. Feb 26, 2021; 9(6): 1433-1438 Published online Feb 26, 2021. doi: 10.12998/wjcc.v9.i6.1433
Unusual presentation of granulomatosis with polyangiitis causing periaortitis and consequent subclavian steal syndrome: A case report
Uiju Cho, Sung-Kyung Kim, Jeong Min Ko, Jinyoung Yoo
Uiju Cho, Department of Pathology, St. Vincent’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, South Korea
Sung-Kyung Kim, Department of Internal Medicine, St. Vincent’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, South Korea
Jeong Min Ko, Department of Radiology, St. Vincent’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, South Korea
Jinyoung Yoo, Department of Hospital Pathology, St. Vincent’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, South Korea
Author contributions: Cho U and Yoo J performed the pathologic examination and diagnosed the patient’s case, reviewed the literature and contributed to manuscript drafting; Kim SK was the patient’s physician and contributed to manuscript drafting; Ko J performed the radiologic analysis of the patient’s case and contributed to manuscript drafting; all authors issued final approval for the version to be submitted.
Informed consent statement: Written informed consent was obtained from the patient.
Conflict-of-interest statement: The authors declare that there is no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Jinyoung Yoo, MD, PhD, Doctor, Professor, Department of Hospital Pathology, St. Vincent’s Hospital, College of Medicine, The Catholic University of Korea, Ji-dong 93, Paldal-gu, Seoul 06591, South Korea. jinyyoo@catholic.ac.kr
Received: October 7, 2020 Peer-review started: October 7, 2020 First decision: December 21, 2020 Revised: December 30, 2020 Accepted: January 8, 2021 Article in press: January 8, 2021 Published online: February 26, 2021
Abstract
BACKGROUND
Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease that involves small-to-medium-sized vessels and forms necrotizing vasculitis with granulomatous inflammation. The formation of a large vessel lesion in GPA patients has been scarcely reported, and it can cause confusion in the diagnosis.
CASE SUMMARY
A 27-year-old man presented with mild left-sided pleuritic chest pain that started one year prior. An imaging study revealed up to 2.5 cm-sized two irregular nodular consolidation nodule in the left lower lobe. Both nodules showed central necrosis. Also, there was a periaortic mass occluding the branching porting of the subclavian artery. He had positive anti-neutrophil cytoplasmic antibodies (ANCAs), but myeloperoxidase-ANCAs and proteinase 3-ANCAs were negative. The patient also developed symptoms of subclavian vein syndrome during the follow-up. Wedge resection of the lung revealed necrotizing vasculitis, destructive parenchymal abscess and surrounding granuloma, and therefore diagnosed of GPA. The patient started on methotrexate and steroid therapy with a relief of symptomatic.
CONCLUSION
Here, we present an unusual manifestation of GPA with periaortitis and consequent subclavian steal syndrome, which has never been previously described. This case alerts us that we should include GPA in the differential diagnosis of large vessel vasculitis as well as subclavian steal syndrome.
Core Tip: Granulomatosis with polyangiitis (GPA) is a systemic process characterized by necrotizing vasculitis and granulomatous inflammation. The large vessel involvement by GPA is not only rare but also causes major diagnostic difficulty. We present a rare case of GPA, showing both lung and periaortic lesion in a male patient. Periaortic lesion of the patient caused vascular occlusion and consequent subclavian vein syndrome. On pathologic examination, the lung nodules had typical histologic features of GPA. This case reminds us that the GPA should not be excluded from the diagnosis because of a large vascular lesion present in an otherwise suspicious setting.
