• Medicine · Mar 2013

    The spectrum of type I cryoglobulinemia vasculitis: new insights based on 64 cases.

    • 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, and Patrice Cacoub.
    • From the Department of Internal Medicine (BT, DS, P. Cacoub), Groupe Hospitalier Pitié-Salpetrière, Assistance Publique Hôpitaux de Paris, Université Pierre et Marie Curie, Paris 6, Paris; Department of Nephrology (AK), Hôpital Européen Georges Pompidou, Paris; Department of Internal Medicine (J-EK), Hôpital Foch, Suresnes; Department of Internal Medicine (GG), CHU, Clermont-Ferrand; Department of Internal Medicine (IM), CHU, Rouen; Department of Internal Medicine, (LB) CHU, Marseille; Department of Nephrology (AL), CHU, Bordeaux; Department of Internal Medicine (ED), CHU, Tours; Department of Hematology (OH), Hôpital Necker-Enfants Malades, Paris; Department of Internal Medicine (LS-M), CHU, Brest; Department of Internal Medicine (P. Cathébras), CHU, Saint-Etienne; Department of Hematology (VL) and Department of Neurology (J-ML), Groupe Hospitalier Pitié-Salpetrière, Paris; Department of Dermatology (PM), CHRU, Lille; Department of Rheumatology (XM), Hôpital Bicêtre, Le Kremlin-Bicêtre; and Department of Dermatology (PS) and Department of Nephrology (EP), Hôpital Tenon, Paris, France.
    • Medicine (Baltimore). 2013 Mar 1; 92 (2): 61-68.

    AbstractType 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.

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