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Le Touquet – Paris-Plage, France

Geri G.,Service de Medecine Interne II | Geri G.,University Pierre and Marie Curie | Terrier B.,Service de Medecine Interne II | Terrier B.,University Pierre and Marie Curie | And 11 more authors.
Annals of the Rheumatic Diseases | Year: 2010

Objective: To evaluate clinical and biological surrogate markers associated with the presence of B cell non-Hodgkin's lymphoma (B-NHL) in patients with hepatitis C virus (HCV) with mixed cryoglobulinaemia (MC) vasculitis. Methods: A total of 104 patients with HCV-MC vasculitis (including 20 with B-NHL) were included. The main clinical and biological markers associated with the presence of B-NHL were evaluated. Results: Patients with B-NHL compared to those without showed higher rates of poor general status (40% vs 16.7%; p=0.032), purpura (90% vs 66.7%; p=0.05), renal (50% vs 28.6%; p=0.11) and cardiac involvement (15% vs 0%; p=0.0006), higher cryoglobulin levels (1.44 g/litre vs 0.67 g/litre; p=0.0004), and lower C4 (0.025 g/litre vs 0.06 g/litre; p=0.001) and γ-globulin levels (5.3 g/litre vs 13.3 g/litre; p<0.0001). The free light chain κ/λ ratio was more frequently abnormal in patients with than without B-NHL (64.3% vs 33.3%, p=0.10). On multivariate analysis, only γ-globulin level was associated with the presence of B-NHL (OR 0.77 (95% CI -0.44 to -0.13), p=0.0006). The optimal cutoff value for γ-globulin level was 9 g/litre, with sensitivity, specificity, positive and negative predictive values for the presence of B-NHL of 75%, 82%, 50% and 93%, respectively. Conclusions: In patients with HCV-MC, a low γ-globulin level (<9 g/litre) is strongly associated with the presence of B-NHL. Source

Lebeaux D.,Service de Medecine Interne II | Lebeaux D.,University Pierre and Marie Curie | Frances C.,University Pierre and Marie Curie | Barete S.,University Pierre and Marie Curie | And 16 more authors.
Rheumatology | Year: 2012

Objective. To analyse therapeutic management of eosinophilic fasciitis (EF). Methods. We reviewed 34 adult patients with biopsy-proven EF. Analyses focused on the therapeutic management, including treatment modalities, responses and associated or predictive factors. Results. Thirty-four patients were included with a diagnosis age of 53 (15) years. They were featured by cutaneous manifestations (88%) including morphoea (41%), myalgia (86%) and hypereosinophilia (85%). Thirty-two patients (94%) were eligible for treatment evaluation and all received CSs as a first-line therapy. Fifteen patients (47%) received methylprednisolone pulses (MPPs) at treatment initiation and 14 patients (44%) received an immunosuppressive drug (ISD), usually MTX (86%), as a second-line therapy. Complete remission was achieved for 69% of patients, remission with disability 19% and failure 12%. A poor outcome was associated with a diagnosis time delay of >6 months [odds ratio (OR) = 14.7] and the lack of MPPs (OR = 12.9). Conclusion. Our study reports new insights into the therapeutic management of EF: (i) CS treatment remains the standard therapy for EF, taken alone or in association with an ISD; (ii) MPPs at initiation of treatment are associated with a better outcome and a lower need of ISD use; (iii) an ISD, usually MTX, might be useful as a second-line therapy, mainly in patients with morphoea-like lesions. Naturally, these practical conclusions should be confirmed by a prospective and multicentre study. © The Author 2011. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. Source

Geri G.,Service de Medecine Interne II | Geri G.,University Pierre and Marie Curie | Saadoun D.,Service de Medecine Interne II | Saadoun D.,University Pierre and Marie Curie | And 11 more authors.
Clinical Rheumatology | Year: 2014

Central nervous system (CNS) angiitis is a rare inflammatory disorder. To date, clinical data are lacking and treatment remains a matter of debate. The aim of this study is to analyse the main characteristics, response to therapy and outcome of 32 patients with CNS angiitis. Single-centre retrospective study in a tertiary centre was made. Diagnosis of CNS angiitis was made by cerebral angiography and/or magnetic resonance angiography and/or CNS biopsy. The main features and outcomes of primary and secondary CNS angiitis were compared and predictive factors of a favourable outcome were searched. Thirty-one patients (median age 45 Q1-Q3 37-54) sex ratio F/M 2.1) were included. Main clinical features were hemiparesis (35.5 %) and headache (29 %). The median CSF protein level was 0.64(0.52-0.81)g/L and was superior to 1 g/L in six cases. CNS magnetic resonance (MR) imaging findings were most frequently ischemic (96.8 %), bilateral (83.9 %), multiple (87.1 %) and supratentorial (96.8 %). The MR angiography was abnormal in all cases. Among the 31 patients in the study, 19 (61.3 %) were diagnosed with primary CNS angiitis. Systemic lupus erythematosus (n =6) and vasculitis (n =4) were the most frequent aetiologies of secondary CNS angiitis. No difference was evidenced between primary and secondary CNS angiitis. Steroids were administered in 79.2 % of treated patients and combined with immunosuppressants in 79.2% of cases. Eight cases of CNS angiitis relapse were noted. CNS angiitis remains a severe illness. Treatment often associated steroids and immunosuppressants, and diagnosis delay is significantly associated with a poorer prognosis. © 2013 Clinical Rheumatology. Source

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