title: The spectrum of type I cryoglobulinemia vasculitis: new insights based on 64 cases.
abstract: 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.
authors: 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.
authors: Benjamin
authors: B
authors: Terrier
authors: Alexandre
authors: A
authors: Karras
authors: Jean-Emmanuel
authors: JE
authors: Kahn
authors: Guillaume
authors: G
authors: Le Guenno
authors: Isabelle
authors: I
authors: Marie
authors: Lucas
authors: L
authors: Benarous
authors: Adeline
authors: A
authors: Lacraz
authors: Elisabeth
authors: E
authors: Diot
authors: Olivier
authors: O
authors: Hermine
authors: Luc
authors: L
authors: de Saint-Martin
authors: Pascal
authors: P
authors: Cathébras
authors: Véronique
authors: V
authors: Leblond
authors: Philippe
authors: P
authors: Modiano
authors: Jean-Marc
authors: JM
authors: Léger
authors: Xavier
authors: X
authors: Mariette
authors: Patricia
authors: P
authors: Senet
authors: Emmanuelle
authors: E
authors: Plaisier
authors: David
authors: D
authors: Saadoun
authors: Patrice
authors: P
authors: Cacoub
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003449:Cryoglobulinemia / Q000150:complications / Q000188:drug therapy / Q000453:epidemiology*; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000150:complications*; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome; D014657:Vasculitis / Q000188:drug therapy / Q000453:epidemiology* / Q000209:etiology