[Membranous glomerulonephritis during primary Gougerot-Sjögren syndrome]. / Glomérulonéphrite extra-membraneuse au cours d'un syndrome de Gougerot-Sjögren primitif.

ABSTRACT

INTRODUCTION: Glomerulonephritis, mainly membranoproliferative or membranous (MG), is observed much less often than interstitial involvement in Sjögren's syndrome (SS). CASE We report a case of MG revealed by thrombosis of the inferior vena cava and of a renal vein in a 40-year-old woman with primary SS, which began with polyarthritis, immune-type lymphadenopathy, and Hashimoto thyroiditis and did not include obvious sicca syndrome. After failure of moderate-dose steroids and then azathioprine, each over separate 9-month periods, the MG responded well within a few weeks to monthly alternation of methylprednisolone and oral cyclophosphamide for 6 months. DISCUSSION: SS may be an underestimated cause of glomerulonephritis, especially membranoproliferative and membranous glomerulonephritis. They should be considered even in the absence of obvious sicca syndrome. Although the prognosis is usually good, renal insufficiency can occur. In cases of MG, if moderate-dose steroids fail, monthly alternation of methylprednisolone and cyclophosphamide for 6 months appears effective and well tolerated, with a low risk of carcinogenicity.