The Spectrum of Type I Cryoglobulinemia Vasculitis

• DOI: 10.1097/md.0b013e318288925c
• Publication Date: 2013-02-21T18:28:49Z
• Authors: Benjamin Terrier; Alexandre Karras; Jean-Emmanuel Kahn; Guillaume Le Guenno; Isabelle Marie; Lucas Benarous; Adeline Lacraz; Elisabeth Diot; Olivier Hermine; Luc de Saint-Martin; Pascal Cathébras; Véronique Leblond; Philippe Modiano; Jean-Marc Léger; Xavier Mariette; Patricia Senet; Emmanuelle Plaisier; David Saadoun; Patrice Cacoub
• Keywords: Cryoglobulins

Type I cryoglobulinemia vasculitis (CryoVas) is considered a life-threatening condition; however, data on the characteristics and outcome are scarce. To analyze presentation, prognosis, efficacy safety of treatments type CryoVas, we conducted French nationwide survey that included 64 patients with CryoVas between January 1995 July 2010: 28 monoclonal gammopathy unknown significance (MGUS) 36 hematologic malignancy.Type was characterized by severe cutaneous involvement (necrosis ulcers) in almost half high serum cryoglobulin levels, contrasting lower frequency glomerulonephritis than expected. The 1-, 3-, 5-, 10-year survival rates were 97%, 94%, 87%, respectively. Compared to MGUS, related malignancy tended be associated poorer prognosis. Therapeutic regimens based alkylating agents, rituximab, thalidomide or lenalinomide, bortezomib showed similar manifestations, clinical response from 80% 86%.Data show prognosis does not seem as poor previously suggested. Besides use interesting alternative options, although exact role each strategy remains defined.