Periarteritis/periaortitis and retroperitoneal fibrosis in IgG4-related disease

IGg4-related disease is a systemic fibroinflammatory disease that is becoming more relevant. It is characterized by a classic triad on histopathological analysis. Cardiovascular involvement does not go beyond its potential aspect to affect the coronary arteries, heart valves, myocardium, pericardium, aorta and peripheral vasculature, being confused with other rheumatological entities that suffer from this involvement. It is therefore essential to recognize the clinical and imaging characteristics that differentiate it from other inflammatory etiologies for clinical suspicion and better action to avoid potentially fatal complications.The existence of new organ-specific criteria may facilitate the diagnosis of this compromise, especially in patients with a high degree of success in obtaining tissue patterns. IgG4-associated vascular compromise can cause life-threatening complications, such as aneurysmal rupture and arterial stenosis; however, details of the clinical features, prediction, and optimal treatment will remain unclear due to the rarity of this condition.