The overlap of ANCA associated vasculitis with Sjögren’s syndrome in elderly male patient: a case report and literature review

Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a rare disease characterized by inflammation of small vessels in various organs, usually affecting the kidneys, lungs, upper respiratory tract, skin, eyes, and peripheral nerves. The typical renal pathological manifestation is necrotizing crescentic glomerulonephritis. Sjögren's syndrome is another relatively uncommon autoimmune disorder characterized by dry eyes and dry mouth. Here, we present a case of overlap syndrome involving AAV with SS in an elderly male patient who demonstrated significant improvement following aggressive treatment with cyclophosphamide, glucocorticoids, hemodialysis, and rituximab. In addition, we also found 49 cases of overlapping syndrome of ANCA combined with SS through literature review and summarized and analyzed them.

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Journal
Frontiers in Medicine
Published
2026-02-24
DOI
https://doi.org/10.3389/fmed.2026.1679800
Primary Topic
Vasculitis and related conditions
Type
article
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article

The overlap of ANCA associated vasculitis with Sjögren’s syndrome in elderly male patient: a case report and literature review

Wenze Jiang, Zhongjie Qu, Yulu Jia, Haoran Zhu et al.
Frontiers in Medicine
Vasculitis and related conditions
article

The overlap of ANCA associated vasculitis with Sjögren’s syndrome in elderly male patient: a case report and literature review

Wenze Jiang, Zhongjie Qu, Yulu Jia, Haoran Zhu, Ruobing Zhu, Keda Lu, Mengting Zhu, Wo Zhang
article en

Abstract

Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a rare disease characterized by inflammation of small vessels in various organs, usually affecting the kidneys, lungs, upper respiratory tract, skin, eyes, and peripheral nerves. The typical renal pathological manifestation is necrotizing crescentic glomerulonephritis. Sjögren's syndrome is another relatively uncommon autoimmune disorder characterized by dry eyes and dry mouth. Here, we present a case of overlap syndrome involving AAV with SS in an elderly male patient who demonstrated significant improvement following aggressive treatment with cyclophosphamide, glucocorticoids, hemodialysis, and rituximab. In addition, we also found 49 cases of overlapping syndrome of ANCA combined with SS through literature review and summarized and analyzed them.

Frontiers in MedicineVol. 13
The Third Affiliated Hospital of Zhejiang Chinese Medical University (CN), Tianjin Medical University (CN)
Good health and well-being
Openalex Percentile: Top 45%
Vasculitis and related conditions
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