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Background <p>Eosinophilic angiocentric fibrosis (EAF) is a rare, tumefactive inflammatory process of the sinonasal and upper respiratory tract and orbit. Numerous histologic mimickers of EAF occur in these locations.</p> Methods <p>A comprehensive literature review focused on EAF and its histologic mimickers was performed.</p> Results <p>This manuscript serves as an overview of the histopathological characteristics of EAF and its histologic mimickers, including granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), sinonasal inflammatory polyps, epithelioid hemangioma, invasive fungal rhinosinusitis, Langerhans cell histiocytosis, rhinoscleroma, inflammatory myofibroblastic tumor, and fibromatosis.</p> Conclusion <p>A correct diagnosis of EAF can be reached by identifying the classic histopathologic features of prominent eosinophilia, lymphoplasmacytic inflammation, and concentric perivascular fibrosis. Additionally, as EAF sometimes lies on the spectrum of IgG4-related disease, immunohistochemistry and serologic studies can be used to aid in diagnosis.</p>

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Eosinophilic Angiocentric Fibrosis: An Uncommon Entity in the Spectrum of IgG4-related Disease

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年5月26日

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  • Sarah E. Gradecki,
  • Edward B. Stelow

摘要

Background

Eosinophilic angiocentric fibrosis (EAF) is a rare, tumefactive inflammatory process of the sinonasal and upper respiratory tract and orbit. Numerous histologic mimickers of EAF occur in these locations.

Methods

A comprehensive literature review focused on EAF and its histologic mimickers was performed.

Results

This manuscript serves as an overview of the histopathological characteristics of EAF and its histologic mimickers, including granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), sinonasal inflammatory polyps, epithelioid hemangioma, invasive fungal rhinosinusitis, Langerhans cell histiocytosis, rhinoscleroma, inflammatory myofibroblastic tumor, and fibromatosis.

Conclusion

A correct diagnosis of EAF can be reached by identifying the classic histopathologic features of prominent eosinophilia, lymphoplasmacytic inflammation, and concentric perivascular fibrosis. Additionally, as EAF sometimes lies on the spectrum of IgG4-related disease, immunohistochemistry and serologic studies can be used to aid in diagnosis.