Large Vessel Vasculitis Primarily Affecting the Lower Extremities: A Case Series and Literature Review

The increasing use of advanced imaging modalities in the diagnosis of large vessel vasculitis (LVV) has occasionally uncovered atypical disease patterns, complicating both diagnosis and management. In this case series, we present cases with LVV predominantly involving the lower extremities (LE) and review case reports from the literature with the aim of discussing the etiology, diagnosis, and therapeutic implication.

LVV patients diagnosed by 18F-fluorodeoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT) with primarily LE involvement were identified from 3 rheumatology departments. Similarly, case reports were identified by literature search using PubMed. Clinical presentation and management of these cases were described and compared.

Sixteen patients were identified (8 from our clinic, 8 case reports). Most patients presented with unspecific symptoms. Only about 60% reported LE symptoms such as claudication, pain, and/or dysfunction of the legs. Only one patient reported typical cranial giant cell arteritis (GCA) symptoms, while half of the case reports had polymyalgic complaints. Treatment was initiated for most patients presenting any symptom or finding typical for GCA and/or polymyalgia rheumatica (PMR), such as cranial symptoms or proximal muscle pain, a positive temporal artery biopsy, or involvement of the aorta and its proximal branches. Spontaneous improvement also occurred, primarily in patients who were either asymptomatic or patients with medium-small vessels LE vasculitis.

Although rare, vasculitis affecting LE should be considered in patients with inflammation and pain and/or stiffness of the LE. Spontaneous improvement may be seen in some patients not presenting with typical symptoms and findings of GCA.