Rituximab for chronic periaortitis without evidence of IgG4-related disease: a long-term follow-up study of 20 patients

Chronic periaortitis (CP) is a rare condition characterised by a peri-aortoiliac fibro-inflammatory tissue. A total of 20%–50% of the cases are immunoglobulin G4 (IgG4)-related, based on histological evidence of IgG4+ plasma cell infiltration (on a background of dense lymphoplasmacytic infiltrates, storiform fibrosis and tissue eosinophilia) and/or increased serum IgG4.1 Glucocorticoids are the first-line therapy for CP.2 However, some...