Aortitis: An Unusual Inflammatory Complication of Systemic Lupus Erythematosus

Abstract

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can involve any organ system. Vasculitides are classified according to the predominant vessel involved such as large vessel, medium vessel, or small vessel vasculitis. Of these, Takayasu arteritis, Behcet s disease, relapsing polychondritis, and immunoglobulin G4 (IgG4)-related disease predominantly involve large vessels. The most common form of vasculitis seen in SLE is small vessel vasculitis. Aortitis in SLE is an extremely rare complication. This is a case report of a 21-year-old female patient with a history of SLE with overlap syndrome of primary biliary cirrhosis and autoimmune hepatitis associated with antiphospholipid syndrome (APS), who presented with a one-week history of left-back burning lumbar pain, radiating to the flank, which increased with changes in position associated with intermittent claudication. In the angiography images and the positron emission tomography (PET) scan, a hypometabolic left para-aortic oval image was noted, corresponding to the presence of a contained hematoma in an abdominal aorta rupture. Later, she underwent vascular surgery and hemodynamics, performing thoracoabdominal aortic reconstruction together with aortorenal bypass and left nephrectomy. Pathology fundings of the left kidney correspond with class IV lupus nephritis, and the resection sample of the thoracoabdominal aneurysm showed a marked thinning and fragmentation of elastic fibers, areas of fibrosis of the wall with severe IgG4 negative lymphoplasmacytic infiltrate in the immunohistochemical study, establishing the diagnosis of aortitis.