Published online Feb 26, 2021. doi: 10.12998/wjcc.v9.i6.1433
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
Processing time: 122 Days and 2.8 Hours
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.
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.
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.