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Management of nonviral mixed cryoglobulinemia vasculitis refractory to rituximab: Data from a European collaborative study and review of the literature.

Authors :
Pouchelon C
Visentini M
Emmi G
le Guern V
Quartuccio L
Samson M
Venhoff N
Briantais A
Casato M
Chatelus E
Chilles M
Cid MC
Diot E
Ebbo M
Faguer S
Hellmich B
Jachiet M
Moulinet T
Perrin F
Quémeneur T
Sinico RA
Terrier B
Source :
Autoimmunity reviews [Autoimmun Rev] 2022 Apr; Vol. 21 (4), pp. 103034. Date of Electronic Publication: 2022 Jan 04.
Publication Year :
2022

Abstract

Background: Glucocorticoids (GCs) plus rituximab (RTX) represent the first-line treatment of nonviral mixed cryoglobulinemia vasculitis (CryoVas). However, data on therapeutic management and outcome of patients refractory to RTX are lacking.<br />Methods: We conducted a European collaborative retrospective multicenter study of patients with nonviral mixed CryoVas refractory to RTX and performed a literature review.<br />Results: Twenty-six original cases and 7 additional patients from the literature were included. All patients but one had type 2 cryoglobulinemia, and causes were autoimmune disease (51%), malignant hemopathy (12%) or essential CryoVas (42%). CryoVas was primary refractory to RTX in 42%, while 58% had an initial response to RTX before immune escape. After RTX failure, patients received a median of 1 (IQR, 1-3) line of treatment, representing 65 treatment periods during follow-up. Main treatments used were GCs in 92%, alkylating agents in 43%, RTX in combination with other treatments in 46%, and belimumab in 17%. Combination of anti-CD20 plus belimumab, alkylating agents alone and anti-CD20 plus alkylating agents provided the highest rates of clinical response in 100% 82% and 73%, respectively, but showed poor immunological response, in 50%, 30% and 38%, respectively. Rates of severe infection were 57%, 9% and 0% in patients receiving anti-CD20 plus belimumab, alkylating agents alone and anti-CD20 plus alkylating agents, respectively.<br />Conclusion: In patients with nonviral mixed CryoVas refractory to RTX, anti-CD20 plus belimumab, and alkylating agents associated or not with anti-CD20, provide the highest rates of clinical response. However, anti-CD20 plus belimumab was frequently associated with severe infections.<br /> (Copyright © 2022 Elsevier B.V. All rights reserved.)

Details

Language :
English
ISSN :
1873-0183
Volume :
21
Issue :
4
Database :
MEDLINE
Journal :
Autoimmunity reviews
Publication Type :
Academic Journal
Accession number :
34995764
Full Text :
https://doi.org/10.1016/j.autrev.2022.103034