Tocilizumab treatment of therapy refractory thoracic aortitis in a patient with retroperitoneal fibrosis

Abstract

In this report, we present the case of a patient with retroperitoneal fibrosis and aortitis. Most authors suggest glucocorticoid treatment as first-line therapy. Further optimal steroid-sparing medication has not been established. Over the years, our patient was treated with mycophenolate mofetil, methotrexate, infliximab, etanercept, azathioprine and several steroid-pulse therapies. Despite of this wide immunosuppressive treatment regular fluor-18-fluorodeoxyglucose positron emission computed tomography scans revealed refractory aortitis of the dilated ascending aorta. Since a growing number of reports support the efficacy of B cell depletion with rituximab in patients with retroperitoneal fibrosis and aortitis, we started medication with rituximab. After nine month of treatment a follow-up fluor-18-fluorodeoxyglucose positron emission computed tomography scan still showed unaffected aortitis. Since the aortitis was refractory to previous immunosuppressive drugs we decided to start medication with tocilizumab. This seemed feasible, since several studies indicated that tocilizumab may be a useful therapy option in patients with inflammatory aortitis and in patients with large vessel vasculitis featuring a positive response upon treatment with tocilizumab. Six we The most recent fluor-18-fluorodeoxyglucose positron emission computed tomography scan after six months of treatment revealed a significant decrease in fluorodeoxyglucose uptake at both the thoracic and abdominal aorta following tocilizumab therapy. To avoid complications like aneurysm or dissection, effective treatment of aortitis related to retroperitoneal fibrosis is necessary. Optimal treatment has not been established, yet. Here, tocilizumab was effective to treat therapy refractory retroperitoneal fibrosis - related aortitis.