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 patients are refractory, frequently relapsing or have contraindications to glucocorticoids. The anti-CD20 monoclonal antibody rituximab proved efficacious in systemic forms of IgG4-related disease (IgG4-RD) including IgG4-related CP,3 but data on IgG4-unrelated CP are scarce.4–6 In this study, we tested rituximab in CP patients without evidence of IgG4-RD who had relapsing/refractory disease or contraindications to standard-dose glucocorticoids. We included patients with active, IgG4-unrelated CP who received rituximab (October 2009 to April 2017). Online supplementary methods describe the diagnostic and follow-up procedures, the definitions of remission and refractory, and the statistical analysis.

Rituximab for chronic periaortitis without evidence of IgG4-related disease: A long-term follow-up study of 20 patients / Urban M.L.; Maritati F.; Palmisano A.; Fenaroli P.; Peyronel F.; Trivioli G.; Ferretti S.; De Biase C.; Grayson P.C.; Pegoraro F.; Prisco D.; Romagnani P.; Emmi G.; Vaglio A.. - In: ANNALS OF THE RHEUMATIC DISEASES. - ISSN 0003-4967. - ELETTRONICO. - 79:(2019), pp. 433-434. [10.1136/annrheumdis-2019-216258]

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

Urban M. L.;Trivioli G.;De Biase C.;Pegoraro F.;Prisco D.;Romagnani P.;Emmi G.;Vaglio A.
2019

Abstract

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 patients are refractory, frequently relapsing or have contraindications to glucocorticoids. The anti-CD20 monoclonal antibody rituximab proved efficacious in systemic forms of IgG4-related disease (IgG4-RD) including IgG4-related CP,3 but data on IgG4-unrelated CP are scarce.4–6 In this study, we tested rituximab in CP patients without evidence of IgG4-RD who had relapsing/refractory disease or contraindications to standard-dose glucocorticoids. We included patients with active, IgG4-unrelated CP who received rituximab (October 2009 to April 2017). Online supplementary methods describe the diagnostic and follow-up procedures, the definitions of remission and refractory, and the statistical analysis.
2019
79
433
434
Goal 3: Good health and well-being
Urban M.L.; Maritati F.; Palmisano A.; Fenaroli P.; Peyronel F.; Trivioli G.; Ferretti S.; De Biase C.; Grayson P.C.; Pegoraro F.; Prisco D.; Romagnani P.; Emmi G.; Vaglio A.
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Utilizza questo identificatore per citare o creare un link a questa risorsa: https://hdl.handle.net/2158/1197249
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