Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Accompanied by IgG4-Tubulointerstitial Nephritis with Underlying Sjögren Syndrome: A Case Report and Review of Literature

Ann Clin Lab Sci. 2021 Nov;51(6):883-889.

Abstract

Objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of autoimmune multisystemic diseases characterized by necrotizing inflammation of small vessels and the presence of circulating ANCA. The prevalence of overlap AAV with other autoimmune diseases was low.

Case report: We report a case of a 54-year-old woman who presented with a 20-year-history of sicca symptoms, the presence of anti-Ro/SS-A, anti-La/SS-B antibodies, myeloperoxidase -ANCA (MPO-ANCA), significant increase of serum IgG4 level, microscopic hematuria, non-nephrotic proteinuria, and progressive renal dysfunction. A renal biopsy showed pauci-immune necrotizing glomerulonephritis with crescents with severe tubulointerstitial nephritis (TIN) which shows extensive infiltration of IgG4-positive plasma cells. Considering these findings and the clinical course, the disease was considered more likely to be MPO-ANCA-associated vasculitis accompanied by IgG4-TIN with underlying primary Sjögren syndrome (pSS).

Conclusion: This report shows a possible unusual disease overlap of MPO-ANCA-associated vasculitis and IgG4-TIN with underlying pSS.

Publication types

  • Case Reports

MeSH terms

  • Acute Kidney Injury / diagnosis
  • Acute Kidney Injury / etiology
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* / diagnosis
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* / etiology
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* / immunology
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* / therapy
  • Antibodies, Antineutrophil Cytoplasmic / blood
  • Antibodies, Antinuclear / blood
  • Biopsy / methods
  • Female
  • Humans
  • Immunoglobulin G / blood
  • Immunoglobulin G4-Related Disease* / complications
  • Immunoglobulin G4-Related Disease* / diagnosis
  • Immunoglobulin G4-Related Disease* / immunology
  • Immunologic Tests / methods
  • Immunosuppressive Agents / administration & dosage*
  • Kidney / immunology
  • Kidney / pathology
  • Kidney Function Tests / methods
  • Middle Aged
  • Nephritis, Interstitial* / diagnosis
  • Nephritis, Interstitial* / etiology
  • Nephritis, Interstitial* / immunology
  • Nephritis, Interstitial* / physiopathology
  • Plasma Cells / immunology
  • Plasma Cells / pathology
  • Sjogren's Syndrome* / complications
  • Sjogren's Syndrome* / diagnosis
  • Sjogren's Syndrome* / immunology

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Antibodies, Antinuclear
  • Immunoglobulin G
  • Immunosuppressive Agents
  • SS-A antibodies