Large Vessel Vasculitis with Leg Involvement - AMJ

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Imaging Uncovers Leg-Predominant Large Vessel Vasculitis

Physician examining lower extremity in patient with suspected large vessel vasculitis

LARGE vessel vasculitis may present as leg pain, and FDG-PET/CT uncovered lower extremity predominant disease in 16 patients today.

Large Vessel Vasculitis Patterns That Defy Expectations

Large vessel vasculitis is typically associated with involvement of the aorta and its major branches, but increasing use of advanced imaging is revealing less typical distributions that can complicate diagnosis and management. In a case series and literature review, investigators described patients with large vessel vasculitis primarily affecting the lower extremities and compared clinic cases with published case reports to clarify clinical patterns and therapeutic implications.

Across three rheumatology departments, eight patients with lower extremity predominant large vessel vasculitis were identified using 18F-fluorodeoxyglucose positron emission tomography with computed tomography (FDG-PET/CT). The authors also included eight published case reports, yielding 16 patients for comparison. Most patients presented with nonspecific symptoms, underscoring how large vessel vasculitis can be missed when classical patterns are absent.

Lower Extremity Predominant Large Vessel Vasculitis and Symptoms

Only about 60% of patients reported lower extremity symptoms such as claudication, pain, or leg dysfunction, meaning a substantial proportion had limited localizing features despite imaging evidence of disease. Typical cranial giant cell arteritis symptoms were uncommon, with only one patient reporting classic cranial features. Polymyalgic complaints were more prominent in the case report subset, appearing in about half of those reports, reinforcing that overlap with polymyalgia rheumatica features may still guide clinical suspicion even when vascular involvement is distal.

Treatment decisions appeared to hinge on whether patients had symptoms or findings typical of giant cell arteritis and or polymyalgia rheumatica. Therapy was most often initiated when patients had cranial symptoms, proximal muscle pain, a positive temporal artery biopsy, or involvement of the aorta and its proximal branches. Notably, spontaneous improvement also occurred, particularly among patients who were asymptomatic or those described as having medium small vessel lower extremity vasculitis.

Overall, the authors conclude that vasculitis affecting the lower extremities, although rare, should be considered in patients with inflammation and lower extremity pain and or stiffness, and that some patients without typical giant cell arteritis features may improve without escalation of therapy.

Reference: Egedal JH et al. Large Vessel Vasculitis Primarily Affecting the Lower Extremities: A Case Series and Literature Review. J Clin Rheumatol. 2026;32(2):74-77.

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