Chronic periaortitis (retroperitoneal fibrosis) concurrent with giant cell arteritis: a case report

Giant cell arteritis is the most common form of large-vessel vasculitides. However, it is probable that extracranial involvement is underdiagnosed in patients with classical giant cell arteritis. In the recent literature most cases of giant cell arteritis have been described in conjunction with aortic aneurysms or dissections. Nonetheless the coexistence of giant cell arteritis and retroperitoneal fibrosis is extremely rare. Here, we describe a case of giant cell arteritis at a very early clinical stage, in a woman with coexistence of retroperitoneal fibrosis.

We report a case of giant cell arteritis at a very early clinical stage, in a 47-year-old Greek woman with coexistence of retroperitoneal fibrosis who was admitted to our hospital with a history of high-grade fever and mild right periumbilical abdominal pain for the past 30 days. In the context of fever of unknown origin, an abdomen computed tomography was ordered. A temporal artery biopsy was also performed because during hospitalization she complained of a headache. Examination of eosin and hematoxylin slides from biopsy specimens of her temporal artery, showed lesions consisting of predominantly lymphocytes, few plasma cells and occasional polymorphonuclear leucocytes. In addition no giant cells were detected in examining biopsies at multiple levels. This was consistent with giant cell arteritis according to the American college of Rheumatology criteria. An abdomen computed tomography revealed the presence of a retroperitoneal soft-tissue mass located anteriorly to the upper infrarenal aorta at the site of the scintigraphic uptake. The computed tomography and magnetic resonance imaging characteristics of the mass were consistent with retroperitoneal fibrosis, and its morphology suggestive of benignity. Our patient started oral prednisolone and was afebrile from day one.

In our experience this is the first case of retroperitoneal fibrosis due to giant cell arteritis occurring at the same time. Involvement of the aorta (aortitis) and its branches has been also observed in a subset of patients with giant cell arteritis. In addition, giant cell arteritis has been associated with a markedly increased risk of aortic aneurysm particularly thoracic aortic aneurysm.