Journal of Clinical Medicine (Sep 2023)

Not Everything Is as It Seems: A Case Series and Overview of Diseases Mimicking Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

  • Eline Houben,
  • Pieter F. de Groot,
  • Yosta Vegting,
  • Josephine M. I. Vos,
  • Erfan Nur,
  • Marc L. Hilhorst,
  • A. E. (Liesbeth) Hak,
  • Arjan J. Kwakernaak

DOI
https://doi.org/10.3390/jcm12196144
Journal volume & issue
Vol. 12, no. 19
p. 6144

Abstract

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Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare heterogeneous disease in which treatment must be initiated early to prevent irreversible organ damage and death. There are several diseases that can mimic AAV, even in the presence of positive ANCA serology and/or histological evidence of vasculitis, as demonstrated in this case series. We reflect on the diagnostic approach of patients with AAV and provide an overview of AAV-mimicking diseases that can be considered in patients with atypical disease presentation or course.

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