RT Journal Article
SR Electronic
T1 Clinical Spectrum, Treatment, and Outcome of Patients with Type II Mixed Cryoglobulinemia without Evidence of Hepatitis C Infection
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 716
OP 722
DO 10.3899/jrheum.100898
VO 38
IS 4
A1 LAURE FOESSEL
A1 JEAN-FRANÇOIS BESANCENOT
A1 GILLES BLAISON
A1 NADINE MAGY-BERTRAND
A1 ROLAND JAUSSAUD
A1 YVES ETIENNE
A1 FRANÇOIS MAURIER
A1 SYLVAIN AUDIA
A1 THIERRY MARTIN
YR 2011
UL http://www.jrheum.org/content/38/4/716.abstract
AB Objective. The clinical spectrum, etiologies, and best therapeutic approaches of type II mixed cryoglobulinemia (MC) not associated with hepatitis C virus (HCV) infection have been poorly described to date. We studied the clinical presentation and outcome of patients with type II MC with no evidence of HCV. Methods. This was a multicenter retrospective study on the clinical presentation and outcome of patients with type II MC without evidence of HCV infection. Only patients with symptomatic MC were included. Results. Thirty-three patients were included (median followup 67.2 mo). Extensive investigations for associated diseases were performed at presentation. MC was related to an autoimmune disease in 14 patients, to a lymphoid malignancy in 4 patients, and to an infectious disease in 2 patients, while MC was classified as essential (primary) in 13. Essential MC tended to be more severe than secondary disease with, in particular, more frequent renal and peripheral nerve involvement. Most patients were treated with steroid with or without immunosuppressive agents, mainly cyclophosphamide. These treatments were unable to induce sustained remission. One patient was successfully treated with lenalidomide. Seven patients with nonmalignant MC were treated with rituximab; 2 had a sustained complete remission, 3 improved greatly but relapsed within 5 months, and 2 experienced a disease flare. Conclusion. An important proportion of non HCV-related type II MC remains essential. Efforts should be made to find other etiologies than HCV, because treatments with steroid and immunosuppressants are not satisfactory, especially in severe forms. In these situations anti-CD20 therapy may present the best option but should be used with caution. New agents such as lenalidomide remain to be evaluated.