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Non-Randall proliferative glomerulonephritis with humps and monotypic IgG deposits in primary Sjögren's syndrome: a
Karine Dahan1, Catherine Albert2, Jean-Benoît Arlet1
1AP-HP, Hôpital Tenon , Service de Néphrologie et Dialyses, F-75020, Paris , France.
Abstract:
Renal involvement is frequent in patients suffering from primary Sjögren's syndrome (pSS). Tubulointerstitial infiltration is the most common renal lesion, while glomerular involvement is rare. We report the case of a 50-year-old woman with pSS who developed renal failure due to an unusual proliferative glomerulonephritis with humps and monotypic IgG1-kappa deposits. Searches for cryoglobulinaemia, anti-double-stranded DNA and anti-neutrophil cytoplasmic antibodies were negative. Serum protein electrophoresis and immunofixation revealed no monoclonal immunoglobulin. Extensive work-up excluded associated infectious, collagen or lymphoproliferative disease. This case adds to the spectrum of pSS-related glomerular disease which is reviewed in depth.
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