Aortic aneurysm as the initial and sole manifestation of giant cell arteritis (GCA) is rare. Here, we present a case of an elderly male with no past history who presented with chest pain. Imaging revealed a thoracic aortic aneurysm with severe aortic regurgitation. He underwent ascending aorta, total arch, aortic root and valve replacements. Histopathological examination of the aorta revealed necrotising aortitis with lymphohistiocytic inflammation and multinucleated giant cells, consistent with GCA. No fungal organisms, acid fast bacilli or spirochetes were detected on histochemical stains and T. pallidum immunohistochemistry, respectively. The plasma cell ratio did not meet the histological diagnostic criteria for IgG4-related disease. The patient had no systemic symptoms. Rheumatology evaluation post-operatively excluded other potential causes, including infective and IgG4-related aortitis, in conjunction with ancillary investigations. Follow-up CT aortogram showed no evidence of active vasculitis. As a result, immunosuppressive therapy was not initiated, and the patient was placed on imaging surveillance. This case underscores the importance of considering vasculitides in patients presenting with isolated, significant thoracic aortic aneurysms, without known connective tissue disorders or atherosclerotic risk factors. In selected cases with no evidence of active disease, conservative management with surveillance may be considered. Long-term follow-up is essential to detect recurrence.
Punjabi et al. (Sun,) studied this question.