Periaortitis

Periaortitis includes a spectrum of rare conditions characterized by the presence of a fibro-inflammatory tissue that commonly involves the abdominal aorta and the common iliac arteries that extends to involve the adjacent structures. Periaortitis is in most cases idiopathic, but in a minority of cases it recognizes infectious, neoplastic, or other etiologies. Idiopathic cases may belong to the spectrum of IgG4-related disease. Radiological examinations are key to the diagnosis, while only in selected cases a diagnostic biopsy is required. The pathogenesis of idiopathic periaortitis is still unclear, but recent findings suggest a systemic autoimmune origin, thus supporting the employment of immunosuppressive therapies.