Successful use of rituximab in granulomatosis with polyangiitis with aortic inflammation.


Division of Rheumatic Diseases, UT Southwestern Medical Center, Dallas, TX, USA. [Email]


Large-vessel inflammation, although rare, has been increasingly recognised as a complication of granulomatosis with polyangiitis (GPA) in recent years. The presentation is highly variable, ranging from an incidental finding to aortic dissection and rupture. Treatment has predominately consisted of a combination of cyclophosphamide and high dose corticosteroids with surgical intervention when indicated. We present the case of a 34-year-old male diagnosed with GPA after presenting with sinus and eye inflammation and the ensuing investigation revealed large vessel involvement that remarkably improved after 6 months of treatment with the combination of rituximab infusions, methotrexate and corticosteroids.