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.
Zhu et al. (Tue,) studied this question.