Presentation: Renal Disease Associated with Mixed Cryoglobulinemia Vasculitis Unrelated to Hcv Infection (2007)

2008 Renal Disease Associated with Mixed Cryoglobulinemia Vasculitis Unrelated to Hcv Infection

Background & Aim: Hepatitis C Virus (HCV) infection represents the major cause of mixed cryoglobulinemia vasculitis (MC)(70-80%). The renal disease associated with this pathologic condition is now well described. The aim of the present study was to analyse demographics, clinical and biological parameters and outcome in patients presenting with renal disease associated with MC not related to HCV infection.
Methods: Records of 20 patients with MC and renal disease with no evidence of HCV infection (by serology and PCR analysis) were retrospectively analysed. Renal biopsy and extensive search for lymphoproliferative disorder were performed at presentation in all patients.
Results: MC was related to primary Sjögren Syndrome (pSS) in nine patients, and to non Hodgkin lymphoma in one patient, while MC was classified as essential in the remaining ten cases. Renal involvement was characterized by microscopic hematuria in all patients, nephrotic range proteinuria in 75%, hypertension in 80%, and renal failure in 85% (mean GFR 46 ml/min/1.73m2). Membranoproliferative glomerulonephritis with subendothelial deposits was observed in all kidney specimens. Skin vasculitis was the main extra-renal manifestation, and peripheral neuropathy appeared to be more frequent in pSS patients (44% vs. 18% in non pSS). In all patients, cryoglobulinemia was classified as type II MC composed of monoclonal IgM kappa and polyclonal IgG. Most patients (17/20) were treated with steroids or immunosuppressive agent, or both. Initial renal remission was observed in 94% of them. However, renal relapse occurred in most patients, with 10% of them reaching end-stage renal disease. Three patients with essential MC developed a B-cell lymphoma 36 to 48 months after the diagnosis of MC. An EBV-induced B-cell lymphoma occurred in only 1/9 pSS patients. Eight (40%) patients died because of cerebral haemorrhage (three pts), infection (one pt), systemic vasculitis (one pt), digestive haemorrhage (one pt) and unknown cause (two pts).
Conclusion: Renal disease associated with MC unrelated to HCV is a rare disease, less severe than HCV-associated MC, with a lower degree of renal failure at presentation and follow-up. Primary Sjögren syndrome is the main condition associated with non HCV-MC in our series. The occurrence of overt B-cell lymphoma during follow-up emphasizes the need to repeat clinical evaluation in those patients.

 P. cacoub, None; D. Saadoun, None; J. sellam, None; M. matignon, None; M. colombat, None; B. mougenot, None; P. vanhille, None; O. moranne, None; P. ronco, None; E. plaisier, None.