Hypocomplementemic urticarial vasculitis syndrome: a rare cause of basilar panacinar emphysema

Abstract

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare condition characterized by an immune complex-mediated disruption of multiple organ systems. Pulmonary involvement is a major cause of morbidity and mortality in patients with HUVS. HUVS has been described by clinical, histological, and laboratory findings. However, the role of radiography in the diagnosis and management of HUVS has not been established. We examine computed tomography findings in a patient with HUVS and explore the potential of thoracic computed tomography imaging to augment the management of HUVS by serving as a diagnostic tool and marker of disease severity.