Mediterr J Rheumatol 2016;27(4):191-3
58-year-old patient with IgG4-related thoracic aortitis
Authors Information
Department of Pathophysiology, Medical School, University of Athens, Athens, Greece

We report a 58-year-old male with a one-month of fever up to 38.5° C and back pain with reflection in the anterior chest wall. Chest CΤ and magnetic resonance angiography (MRA) revealed thickening of the descending thoracic aorta throughout its length and laboratory tests showed elevated serum immunoglobulin IgG4. The patient was diagnosed with noninfectious IgG4-related thoracic aortitis. He was treated with corticosteroids and Azathioprine, and 3 months later, the patient was asymptomatic with normal inflammatory markers, reduction of IgG4 serum levels and with no findings on imaging with MRA.

Cases of IgG4-related thoracic aortitis have been currently described in literature. This rather new entity accounts for around 9% of noninfectious aortitis. As in our case, IgG4-related disease should be considered in any patient found to have aortitis or periaortitis. An early diagnosis and treatment is essential for the survival of such patients.