Emergence of Smoldering ANCA-associated Glomerulonephritis during the Clinical Course of Mixed Connective Tissue Disease and Sjögren's Syndrome

Intern Med. 2018 Jun 15;57(12):1757-1762. doi: 10.2169/internalmedicine.9844-17. Epub 2017 Dec 21.

Abstract

A 67-year-old woman presented with hematuria and proteinuria 16 and 11 months ago, respectively. She had been followed up as mixed connective tissue disease and Sjögren's syndrome for over 19 years. Blood chemistry showed no elevated serum creatinine or C-reactive protein but did reveal myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) of 300 U/dL. A kidney biopsy showed pauci-immune focal necrotizing glomerulonephritis. She was treated with prednisolone and rituximab, resulting in normal urinalysis and decreased MPO-ANCA. The complication of ANCA-associated glomerulonephritis should not be overlooked when abnormal urinalysis findings appear in the course of connective tissue disease, irrespective of the presence of rapidly progressive glomerulonephritis.

Keywords: ANCA-associated glomerulonephritis; Sjögren's syndrome; mixed connective tissue disease.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Antibodies, Antineutrophil Cytoplasmic / metabolism*
  • Female
  • Glomerulonephritis / complications*
  • Glomerulonephritis / drug therapy
  • Glomerulonephritis / immunology
  • Humans
  • Mixed Connective Tissue Disease / complications*
  • Mixed Connective Tissue Disease / drug therapy
  • Peroxidase / immunology
  • Prednisolone / therapeutic use
  • Proteinuria / complications
  • Rituximab / therapeutic use
  • Sjogren's Syndrome / complications*
  • Sjogren's Syndrome / drug therapy

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Rituximab
  • Prednisolone
  • Peroxidase