Open AccessThe Spectrum of Type I Cryoglobulinemia Vasculitis
Author(s) -
Benjamin Terrier,
Alexandre Karras,
JeanEmmanuel Kahn,
G. Le Guenno,
I. Marie,
Lucas Benarous,
Adeline Lacraz,
Élisabeth Diot,
Olivier Hermine,
Luc de Saint Martin,
P. Cathébras,
Véronique Leblond,
P. Modiano,
JeanMarc Léger,
Xavier Mariette,
Patricia Senet,
Emmanuelle Plaisier,
David Saadoun,
Patrice Cacoub
Publication year - 2013
Publication title -
medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.59
H-Index - 148
eISSN - 1536-5964
pISSN - 0025-7974
DOI - 10.1097/md.0b013e318288925c
Subject(s) - medicine , cryoglobulinemia , rituximab , bortezomib , vasculitis , malignancy , cryoglobulin , gastroenterology , thalidomide , dermatology , immunology , lymphoma , multiple myeloma , hepatitis c virus , disease , virus
Type I cryoglobulinemia vasculitis (CryoVas) is considered a life-threatening condition; however, data on the characteristics and outcome are scarce. To analyze the presentation, prognosis, and efficacy and safety of treatments of type I CryoVas, we conducted a French nationwide survey that included 64 patients with type I CryoVas between January 1995 and July 2010: 28 patients with monoclonal gammopathy of unknown significance (MGUS) and 36 with hematologic malignancy.Type I monoclonal CryoVas was characterized by severe cutaneous involvement (necrosis and ulcers) in almost half the patients and high serum cryoglobulin levels, contrasting with a lower frequency of glomerulonephritis than expected. The 1-, 3-, 5-, and 10-year survival rates were 97%, 94%, 94%, and 87%, respectively. Compared to MGUS, type I CryoVas related to hematologic malignancy tended to be associated with a poorer prognosis. Therapeutic regimens based on alkylating agents, rituximab, thalidomide or lenalinomide, and bortezomib showed similar efficacy on vasculitis manifestations, with clinical response rates from 80% to 86%.Data from the CryoVas survey show that the prognosis of type I CryoVas does not seem to be as poor as previously suggested. Besides alkylating agents, the use of regimens based on rituximab, thalidomide or lenalinomide, and bortezomib are interesting alternative options, although the exact role of each strategy remains to be defined.