Unusual presentation of granulomatosis with polyangiitis causing periaortitis and consequent subclavian steal syndrome:A case report  

作　　者：Uiju Cho Sung-Kyung Kim Jeong Min Ko Jinyoung Yoo 

机构地区：[1]Department of Pathology,St.Vincent’s Hospital,College of Medicine,The Catholic University of Korea,Seoul 06591,South Korea [2]Department of Internal Medicine,St.Vincent’s Hospital,College of Medicine,The Catholic University of Korea,Seoul 06591,South Korea [3]Department of Radiology,St.Vincent’s Hospital,College of Medicine,The Catholic University of Korea,Seoul 06591,South Korea [4]Department of Hospital Pathology,St.Vincent’s Hospital,College of Medicine,The Catholic University of Korea,Seoul 06591,South Korea

出　　处：《World Journal of Clinical Cases》2021年第6期1433-1438,共6页世界临床病例杂志

摘　　要：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.

关 键 词：Granulomatosis with polyangiitides Wegener granulomatosis Systemic vasculitis Subclavian steal syndrome PERIAORTITIS Case report 

分 类 号：R593.2[医药卫生—内科学] R781.42[医药卫生—临床医学]