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Updated: Jan 18, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Eosinophilic Angiocentric Fibrosis: An Uncommon Entity in the Spectrum of IgG4-related Disease
Sarah E Gradecki1, Edward B Stelow2
1Department of Pathology, University of Virginia, 1215 Lee Street, PO Box 800214, Charlottesville, VA, 22908, USA.
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.
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