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Kidney Week

Abstract: PO1949

Overlap Syndrome of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis and IgG4-Related Disease: Distinct Clinicopathologic Clues for Precise Diagnosis

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials


  • Liang, Peifen, Department of Nephrology, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
  • Yang, Qiongqiong, Department of Nephrology, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
  • Chen, Wenfang, Department of Pathology, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China
  • Yue, Shuling, Guangzhou KingMed Center for Clinical Laboratory Co., Ltd, Guangzhou, China
  • Han, Qianqian, Department of Nephrology, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China

Both antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and IgG4-related disease (IgG4-RD) are multi-system inflammatory disorders. The coexistent of both diseases present the possibility of a new overlap syndrome which leads to different treatment and outcome. In this study, the symptomatic and pathological concurrence of AAV and IgG4-RD is investigated to explore the possibility and clinicopathologic clues to the diagnosis of this overlapped syndrome.


A case of a 67-year-old man in our hospital who exhibited the characteristics of both AAV and IgG4-RD was presented. The treatment response and outcome of the case were followed up for the next 15 months. Then, a systematic literature review of the overlap syndrome of AAV and IgG4-RD was performed on PUBMED database from 1976 until January 2020.


Mild hematuria with rapid progressive renal failure of the patient was observed while renal biopsy revealed pauci-immune crescentic glomerulonephritis, especially with IgG4-related tubulointerstitial nephritis. Glucocorticoids combined with cyclophosphamide therapy led to partial remission. Literature review of 52 patients met both AAV and IgG4-RD criteria as overlap syndrome and four common clinicopathologic features were identified. First, atypical clinical and laboratory manifestations were characteristics of this entity. Second, positive MPO-ANCA are more common. Third, tissue samples showed overlapping histological patterns when kidneys were involved. Fourth, the combination of glucocorticoids and immunosuppressive therapy was often required and led to a remission within 3 months.


AAV may overlap with IgG4-RD while presenting atypical manifestations. Four common clinicopathologic characteristics could be used as specific clues to the diagnosis of overlap syndrome.