Aortitis as a Harbinger of Esophageal Cancer

Abstract

Aortitis is a rare diagnosis that requires a high index of suspicion due to nonspecific symptoms and its multiplicity of etiologies. An 80-year-old man, independent in activities of daily living (ADLs), had three consecutive hospitalizations in three months for fever, general malaise, anorexia associated with arthritis of the hands and feet with the inability to walk. Inflammatory markers were increased without a focus of infection identified. Upper digestive endoscopy (UDE), colonoscopy, blood cultures, thoracoabdominal-pelvic CT and transthoracic (TT) echocardiogram were performed without changes, with discharge for consultation after demonstrating apyrexia. At the patient second hospitalization for fever and arthritis, a transesophageal echocardiogram was performed that showed the presence of multiple complex atherosclerotic plaques, with associated thrombi in all segments of the aorta with a suspicious mass of vegetation on the aorta. Thoracic-abdominopelvic CT demonstrated calcified atheromatosis of the entire aorta with para-aortic nodes; MRI showed aortic thickening; and autoimmunity study was negative. Aortitis was the working diagnosis of possible infectious etiology and anticoagulation and antibiotic therapy were started. Fever recurred and a third admission led to a working diagnosis of inflammatory, non-infectious aortitis. The patient responded well to empiric corticosteroids. The patient followed up in consultation, remained asymptomatic under a low dose of corticosteroids with negative temporal artery biopsy. In the sixth month, he repeated UDE due to dysphagia, which showed the presence of esophageal neoformation with the histological diagnosis of squamous cell carcinoma, maintaining on the CT as alterations in the aorta. This is an unusual case of aortitis associated with arthritis with improvement after corticosteroids, which interestingly occurred before the progression of esophageal cancer. In hindsight, we think this may have been a large vessel paraneoplastic vasculitis that preceded the detection of esophageal squamous cell carcinoma.

Keywords: aortitis; arthritis; esophageal carcinoma; large vessel paraneoplastic vasculitis; paraneoplastic syndrome; syndrome of fever of unknown origin.