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A 54–year-old previously asymptomatic woman was presented with acute onset of chest pain and bilateral lower limb weakness. She denied any fever, headache, jaw claudication, visual defects or scalp tenderness. On clinical examination, she had low grade fever, palpable peripheral pulses and bilateral lower extremity weakness (muscle strength: 2/5) with preserved tendon reflexes and sensation. Laboratory work-up showed elevated CRP (110 mg/L, normal < 5 mg/L) and ESR (140 mm/h), normocytic anemia (Hb=9 g/dL) and thrombocytosis (PLT=510.000/μL). Evaluation for acute coronary syndrome and pulmonary embolism was negative. Chest CT showed aortic dissection extending from the descending thoracic aorta after the left subclavian artery to the celiac axis (type B). Thoracic and abdominal aortic MRA confirmed the dissection and revealed aortic wall thickening, while a PET-CT scan showed increased aortic wall uptake of ¹⁸FDG in the areas of dissection as well as at the aortic root and ascending aorta, consistent with aortitis. Temporal artery biopsy and serum IgG4 levels were normal. The patient was diagnosed with aortitis and anterior spinal syndrome due to presumed occlusion of the Adamckiewitz artery, leading to her neurological impairment. She was initially treated with IV methylprednisolone pulses followed by daily oral corticosteroids that resulted in rapid clinical improvement and normalization of acute phase reactants. Six months later, she was fully ambulatory without any assistance.

There is an increasing number of reports in the recent literature of typical and atypical cases of non-infectious inflammatory aortitis as a cause of aortic aneurysm formation¹ and dissection.² Aortic wall inflammation can be isolated or found in systemic diseases that involve large vessels (giant-cell arteritis, Takayasu arteritis or IgG4-RD). A dissecting thoracic aneurysm in relatively young patients, with atypical features such as in this case, should raise the suspicion of aortitis that could be proven with appropriate imaging and treated appropriately. In that respect, the appropriate use of modern imaging modalities such as PET-CT scan which are very sensitive in identifying vascular inflammation represents an extremely useful tool in early diagnosis and initiation of treatment in these patients.

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