Idiopathic IgG4-negative perivascular fibrosis with extensive thoracic aortic, coronary artery, and supra-aortic branch involvement: A case report.

Perivascular fibrosis is an uncommon fibroinflammatory disorder characterized by circumferential soft-tissue thickening surrounding the aorta and its major branches. Involvement of the thoracic region and supra-aortic branches is less common, potentially leading to imaging studies that can resemble large-vessel vasculitis, inflammatory pseudoaneurysms, or lymphoproliferative disorders. In this report, we discuss a case involving a 55-year-old male with long-standing retroperitoneal fibrosis and chronic occlusion of the infrarenal aorta. The condition also exhibited extensive involvement of the thoracic aorta, coronary artery, and supra-aortic branches. Coronary artery stenosis that resulted in ST-elevation myocardial infarction demanding percutaneous intervention and surgical revascularization was a component of disease progression over 20-year follow-up. This case highlights the essential role of multimodal imaging techniques in differentiating perivascular fibrosis from primary large-vessel vasculitis and malignancies, particularly in patients who are IgG4-negative with considerable thoracic involvement.