Rituximab therapy for chronic periaortitis

Chronic periaortitis (CP) is a rare condition, hallmarked by periaortic fi bro-infl ammatory tissue which often causes ureteral obstruction, and encompasses idiopathic retroperitoneal fi brosis and infl ammatory abdominal aortic aneurysm (IAAA). CP usually responds to glucocorticoids, but some patients may be steroid-refractory or not tolerate standard glucocorticoid doses. For such cases, valid therapeutic alternatives are lacking. Combinations of prednisone and immunosuppressants (eg, cyclophosphamide, mycophenolate mofetil) are not of proven superiority to prednisone alone, and their effectiveness in refractory CP is unknown. Because B cells abundantly infi ltrate CP lesions, and CP is often associated with autoimmune diseases, we used rituximab in two patients with CP, one refractory to conventional treatments, and the other with contraindications to standard-dose glucocorticoids.