B-CELL LYMPHOPROLIFERATION IN PATIENTS WITH CHRONIC HEPATITIS C VIRUS INFECTION ASSOCIATED WITH SJÖGREN SYNDROME

M. Ramos-Casals, L. La Civita , S. De Vita , R. Solans , M. Luppi , F. Medina , P. Caramaschi , P. Fadda , G. De Marchi , A. Lopez-Guillermo , J. Font And the SS-HCV Study Group; Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain, Internal Medicine Unit, S. Martino Hospital, Oristano, Clinic of Rheumatology, University of Udine, Udine, Italy, Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain, Department of Oncology and Hematology, University of Modena, Modena, Italy, Department of Rheumatology, Centro Medico Nacional Siglo XXI, Mexico DF, Mexico, Department of Clinical and Experimental Medicine, University of Verona, Verona, Italy, Department of Hematology, Hospital Clinic, Barcelona, SpainObjectives: To characterize the clinical and immunologic patterns of expression of patients with hepatitis C virus (HCV) infection and associated Sjögren syndrome (SS) who developed B-cell lymphoma.Methods: Fifteen international reference centers constituted a multicenter study group with the aim of creating a registry of patients with HCV-SS who developed B-cell lymphoma. A protocol form was used to record the main characteristics of SS, chronic HCV infection and B-cell lymphoma.Results: Twenty-five HCV-SS patients with B-cell lymphoma were included in the registry. There were 22 (88%) women and 3 (12%) men, with a mean age of 55 years at SS diagnosis, 58 years at diagnosis of HCV infection and 61 at lymphoma diagnosis. The main extraglandular SS manifestations were cutaneous vasculitis in 15 (60%) patients and peripheral neuropathy in 12 (48%), while the main immunologic features were RF+ in 24 (96%) and type II cryoglobulins in 20 (80%). The main histotypes were MALT lymphoma in 11 (44%) cases, diffuse large B-cell lymphoma in 6 (24%) and follicular center cell lymphoma in 6 (24%). Fifteen (60%) patients had an extranodal primary location, most frequently in the parotid gland (n=5), liver (n=4) and stomach (n=4). Twelve (52%) out of 23 patients died after a median follow up from the time of lymphoma diagnosis of 4 years, with lymphoma progression being the most frequent cause of death. Survival differed significantly between the main types of B-cell lymphoma (log-rank, p=0.0008).Conclusion: SS-HCV patients with B-cell lymphoma are clinically characterized by a high frequency of cryoglobulinemic-related manifestations, including vasculitis, RF and mixed type II cryoglobulins, together with a predominance of MALT lymphomas involving organs in which HCV replicates (exocrine glands, liver and stomach).Citation: Ann Rheum Dis, volume 65, supplement II, year 2006, page 552Session: Infection-related rheumatic diseases