Steroid responsive aortitis
1 Department of Cardiology, St Antonius Hospital, Koekoekslaan 1, PO Box 2500, 3430 EM, Nieuwegein, The Netherlands
2 Department of Internal Medicine, Meander Medical Centre, Amersfoort, The Netherlands
3 Department of Nuclear Medicine, Meander Medical Centre, Amersfoort, The Netherlands
4 Department of Cardio-thoracic Surgery, St Antonius Hospital, Nieuwegein, The Netherlands
5 Department of Cardiology, Meander Medical Centre, Amersfoort, The Netherlands
* Corresponding author. Tel: +31 30 6093100; fax: +31 30 6092277. E-mail address: avanoostrom{at}hotmail.com
A 57-year-old woman with a history of nephrolithiasis, hypertension, and hypercholesterolaemia presented with a 4-day history of episodic back pain localized between the scapulae. Blood pressure was 155/60 mmHg on both arms and temperature was 36.2°C. Cardiopulmonary, arterial pulsation, and abdominal examinations were unremarkable without inducible back pain. C-reactive protein (C-reactive protein: 40 mg/dL) and erythrocyte sedimentation rate (ESR: 70 mm/h) were elevated. Chest CT scan with contrast demonstrated ascending aorta dilatation (40 mm diameter), aortic arch calcification, and wall thickening of the entire aorta. Assuming a mural thrombus or intramural haematoma, patient was treated with analgetics, anti-hypertensive drugs, and immobilization. Her condition worsened with persistent back pain, low-grade fever (<38.5°C), and ongoing C-reactive protein and ESR elevation (maximum 180 mg/dL and 110 mm/h); blood cultures were negative. After an overnight fast, a fused CT and 18F-labelled deoxyglucose (FDG) positron emission tomography (PET) scan showed pathological FDG uptake in the entire aorta, suggestive of aortitis (upper panels). Autoantibodies against antinuclear antigens and antineutrophil cytoplasmatic antibodies were negative, and lues was excluded. Patient was treated with prednisone 60 mg/day for 2 months. A follow-up PET–CT scan demonstrated resolution of the increased aortic FDG uptake (lower panels), in addition to a reduced C-reactive protein (5 mg/dL) and ESR (36 mm/h), and relieve of her symptoms, suggestive of an inflammatory origin of the aortitis. Our patient did not meet the criteria for giant cell- and Takayasu arteritis. The diagnose was set for a steroid responsive idiopathic aortitis with aorta dilatation. Dose reduction of prednisone will be attempted, her prognosis is unknown.
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