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

## TL;DR

This paper reports a rare case of a man with two autoimmune diseases, AAV and Sjögren’s syndrome, and finds similar cases in the literature.

## Contribution

The paper presents a case of AAV overlapping with Sjögren’s syndrome and reviews 49 similar cases from the literature.

## Key findings

- An elderly male patient with AAV and Sjögren’s syndrome showed improvement with aggressive treatment.
- A literature review identified 49 additional cases of overlapping AAV and Sjögren’s syndrome.
- The combination of these two autoimmune diseases is rare and requires tailored treatment approaches.

## 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.