Clinical characterization of aortitis and periaortitis: study of 134 patients from a single university hospital.

Abstract

Aortitis and periaortitis refer to the inflammation of the aortic wall and the surrounding tissues. Both conditions are associated with various diseases and express nonspecific manifestations. Early diagnosis and treatment are crucial to improve the prognosis of the disease. This study aimed to assess the causes and main clinical features of aortitis and periaortitis in patients from a single centre in Spain. Observational, retrospective study of patients diagnosed with aortitis or periaortitis at a Spanish referral center over the last decade. 134 patients (87 female; mean age of 55.1 ± 9.1 years) were recruited, 132 of which had aortitis and two periaortitis. Aortitis was associated with giant cell arteritis (n = 102), Takayasu's arteritis (n = 6), IgG4-related disease (n = 6), infectious diseases (n = 3), malignancy (n = 1), drugs (n = 1), isolated aortitis (n = 1), and other immune-mediated inflammatory diseases (IMIDs) (n = 12). IMIDs included were Sjögren's syndrome (n = 2), sarcoidosis (n = 2), rheumatoid arthritis (n = 2), axial spondyloarthritis (n = 2), inflammatory bowel disease (n = 1), primary biliary cirrhosis (n = 1), idiopathic pulmonary fibrosis (n = 1), and polyarteritis nodosa (n = 1). Periaortitis was due to idiopathic retroperitoneal fibrosis in both cases. Imaging techniques used for diagnosis included ¹⁸F-FDG PET/CT scan (n = 133), CT-angiography (n = 44), and/or MRI-angiography (n = 33). Polymyalgia rheumatica (52.2%) and asthenia (53.7%) were the most common manifestations, followed by limb claudication (23.9%) and inflammatory back pain (26.9%). Acute-phase reactants were typically increased. Aortitis is a common condition and may be associated with multiple non-infectious diseases. Its clinical presentation is often unspecific, requiring a high level of suspicion to get an early diagnosis and treatment.