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Background <p>Antineutrophil cytoplasmic antibodies (ANCA)-associated small-vessel vasculitis (AAV) and large-vessel vasculitis generally occur separately. An overlap between the two entities is rare and poses diagnostic and therapeutic challenges.</p> Case presentations <p>We present two cases of an overlap syndrome involving AAV and giant cell arteritis (GCA). In a&#xa0;76-year-old female patient with typical clinical presentation and imaging evidence of GCA of the temporal artery, high-titer myeloperoxidase-ANCA (MPO-ANCA) were detected. Glomerular hematuria subsequently appeared and a&#xa0;renal biopsy confirmed pauci-immune glomerulonephritis. An overlap syndrome was diagnosed and remission induction treatment with glucocorticoids (GC) and cyclophosphamide (CYC) followed by rituximab (RTX) was initiated.</p> <p>An 85-year-old female patient with known MPO-ANCA-associated vasculitis, previously histologically confirmed in the lungs, developed a&#xa0;relapse with renal involvement after 5&#xa0;years of remission. At the same time, GCA was confirmed by imaging and histology, but without typical clinical symptoms. Despite remission of the AAV under treatment with GC, CYC, RTX and avacopan, large-vessel involvement persisted on positron emission tomography-computed tomography (PET-CT), necessitating an adjustment of treatment to include methotrexate (MTX) and later tocilizumab.</p> Conclusion <p>These two cases illustrate the rare coexistence of AAV and GCA. They underscore the importance of a&#xa0;comprehensive diagnostic re-evaluation in cases of recurrence as well as the need for individualized treatment decisions.</p>

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„Vaskulitis Overlap“ – ein diagnostisches und therapeutisches Dilemma

  • 期刊论文

  • 发表日期：

    2026年8月5日

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  • F. Tissi,
  • A. U. Marchetti,
  • S. A. Fuss,
  • J. Trachsler,
  • C. Lechtenböhmer,
  • S. Höller,
  • C. Iking-Konert

摘要

Background

Antineutrophil cytoplasmic antibodies (ANCA)-associated small-vessel vasculitis (AAV) and large-vessel vasculitis generally occur separately. An overlap between the two entities is rare and poses diagnostic and therapeutic challenges.

Case presentations

We present two cases of an overlap syndrome involving AAV and giant cell arteritis (GCA). In a 76-year-old female patient with typical clinical presentation and imaging evidence of GCA of the temporal artery, high-titer myeloperoxidase-ANCA (MPO-ANCA) were detected. Glomerular hematuria subsequently appeared and a renal biopsy confirmed pauci-immune glomerulonephritis. An overlap syndrome was diagnosed and remission induction treatment with glucocorticoids (GC) and cyclophosphamide (CYC) followed by rituximab (RTX) was initiated.

An 85-year-old female patient with known MPO-ANCA-associated vasculitis, previously histologically confirmed in the lungs, developed a relapse with renal involvement after 5 years of remission. At the same time, GCA was confirmed by imaging and histology, but without typical clinical symptoms. Despite remission of the AAV under treatment with GC, CYC, RTX and avacopan, large-vessel involvement persisted on positron emission tomography-computed tomography (PET-CT), necessitating an adjustment of treatment to include methotrexate (MTX) and later tocilizumab.

Conclusion

These two cases illustrate the rare coexistence of AAV and GCA. They underscore the importance of a comprehensive diagnostic re-evaluation in cases of recurrence as well as the need for individualized treatment decisions.