Abstract: PO1949
Overlap Syndrome of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis and IgG4-Related Disease: Distinct Clinicopathologic Clues for Precise Diagnosis
Session Information
- Glomerular Diseases: Clinical, Outcomes, and Trials - 3
October 22, 2020 | Location: On-Demand
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1203 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- 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
Background
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.
Methods
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.
Results
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.
Conclusion
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.