Sjoegren's syndrome complicated by MPO-ANCA positive necrotizing and crescentic glomerulonephritis

Abstract

  Although Sjoegren's syndrome (SjS) is occasionally complicated with tubulointerstitial nephritis as one of the extraglandular manifestations, the association of crescentic glomerulonephritis is not common. We report here a female patient with SjS complicated with myeloperoxidase-specific anti-neutrophil cytoplasmic antibodies positive rapidly progressive glomerulonephritis. Renal biopsy revealed necrotizing and crescentic glomerulonephritis in addition to tubulointerstitial nephritis. She developed progressive renal failure, but this was improved by steroid therapy including methylpredonisolone pulse therapy.