A Case Report and Systematic Review of Eosinophilic Angiocentric Fibrosis of the Paranasal Sinuses

Neal, Molly Heft; Rowan, Nicholas R.; Willson, Thomas D.; Wang, Eric; Lee, Stella E.

doi:10.1177/0003489417696510

Cited by 12 publications

(10 citation statements)

References 40 publications

(88 reference statements)

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“…Contrary to the view that majority of EAF cases lie on the spectrum of IgG4‐RD, fewer than half (six of 20, 30%) of the cases assessed in this study met the 2019 ACR/EULAR classification criteria for IgG4‐RD. Seven cases 7,12–14,18,19 (including one from the present paper) did not meet the initial entry criterion of characteristic organ involvement. Of the remaining seven cases, a further five cases 2,3,7,11,20 were noted to lack sufficient data, including serum IgG4 levels and/or IHC investigations for IgG4.…”

Section: Discussionmentioning

confidence: 85%

Eosinophilic angiocentric fibrosis and immunoglobulin 4‐related disease revisited

et al. 2022

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Eosinophilic angiocentric fibrosis (EAF) has been postulated to lie on the spectrum of IgG4‐related disease (IgG4‐RD) in a study reported in 2011. However, the aetiology of EAF remains controversial, despite increasing literature reporting IgG4‐RD targeted investigations in those studies. This review aims to formally evaluate the relationship between EAF and IgG4‐RD using the latest 2019 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification scheme for IgG4‐RD. A literature search was performed on the PubMed database for all studies describing patients diagnosed with EAF. Additionally, two EAF cases were retrospectively identified from our institution and presented. The demographics, clinical presentation, histological and serological data of each patient were analysed. A total of 20 cases were evaluated, including 18 patients from 14 articles in the English literature and two cases from our institution. Six of 20 (30%) patients with EAF met the criteria for IgG4‐RD with the 2019 ACR/EULAR classification scheme. This represents the first attempt at formally evaluating the relationship between EAF and IgG4‐RD using a validated set of classification criteria. Using the currently available data, a small proportion of patients with EAF met the criteria for IgG4‐RD. However, various limitations of this study suggest that further efforts at disease characterization of EAF may be required.

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Section: Discussionmentioning

confidence: 85%

Eosinophilic angiocentric fibrosis and immunoglobulin 4‐related disease revisited

et al. 2022

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“…Diffuse inflammatory lesions involving the orbit or sinus take longer to treat, recur more frequently, and often require multiple operations. Studies have shown that 62% of sinus EAF cases undergo complete resection, with a recurrence rate of 20% [ 20 ]. Therefore, this disease requires close follow-up and observation and timely evaluation of recurrence.…”

Section: Discussionmentioning

confidence: 99%

A rare lumpy inflammatory lesion of the orbit: a unique case report

Ren

et al. 2023

Diagn Pathol

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Background Eosinophilic angiocentric fibrosis (EAF) is a rare inflammatory lesion, especially in orbit. EAF is believed to be related to IgG4-related disease (IgG4-RD), but the clinical manifestations of systemic involvement are relatively rare and easy to be confused with tumors or other inflammatory diseases. Histopathological examination is the most important way of its diagnosis and differentiation. Case presentation We presented a 55-year-old female patient presented with recurrent swelling of the right lower eyelid for more than 2 months. The pathological diagnosis was EAF. Positive immunostaining for IgG, CD34, κ, and λ, while negative immunostaining for IgG4. Conclusions Complete surgical resection is the preferred treatment, histopathological examination is the main diagnostic standard.

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“…Few EAF cases have been reported in the literature with a full evaluation of IgG4-RSD criteria [ 5 , 7 , 11 , 22 , 24 ] and only a few of them have met the criteria to be considered part of IgG4-RSD. Some studies have hypothesized that the similarity between EAF and IgG4-RSD is more striking in the early stages of the disease, while in the later stages it becomes more difficult to show both IgG4 + plasma cells in the perivascular infiltrate and high IgG4 serum levels [ 9 , 10 ]. Table 2 summarizes the previously reported cases of EAF and their association with IgG4 serum levels.…”

Section: Discussionmentioning

confidence: 99%

“…EAF clinically presents as a tumefactive lesion, more often occurring in the upper respiratory tract, the sinonasal region, and the orbit, frequently causing progressive and prolonged airway obstruction [ 3 , 4 , 5 , 6 , 7 , 8 ]. Septal disease with the lateral wall, paranasal sinuses, and subglottic area involvement may also be seen [ 9 , 10 , 11 , 12 ]. Until now, to the best of our knowledge, just a single case of cutaneous involvement has been described [ 13 ] as an asymptomatic nodule.…”

Section: Introductionmentioning

confidence: 99%

Eosinophilic Angiocentric Fibrosis of the Nasal Cavities: A Report of an Uncommon Lesion with Emphasis on the Etiology and Differential Diagnosis

Farina

Broggi

Federico³

et al. 2022

Medicina

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Background and Objectives: Eosinophilic angiocentric fibrosis (EAF) is an indolent but sometimes locally destructive lesion with a predilection for the sinonasal tract. Although it was first described in 1983, its etiology remains unknown. Some authors initially attributed EAF to trauma, hypersensitivity, and/or surgical manipulation, while it has been recently suggested to include EAF within the spectrum of IgG4-related systemic diseases. Materials and Methods: We report an uncommon case of idiopathic EAF in a 76-year-old male who developed two bilateral tumefactive masses in the nasal cavities. Results: As the histological examination showed a subepithelial proliferation of fibroblasts along with sclero-hyaline fibrosis around small-sized vessels (an “onion skin-like” pattern) and an eosinophils-rich inflammatory infiltrate, a diagnosis of EAF was rendered. The differential diagnosis included granuloma faciale, Wegener’s granulomatosis, and Churg–Strauss syndrome. Conclusions: Pathologists should be aware of the possibility that this lesion can be part of the wide spectrum of IgG4-related systemic diseases by performing IgG4 investigations to assess adherence to IgG4-related systemic disease criteria.

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A Case Report and Systematic Review of Eosinophilic Angiocentric Fibrosis of the Paranasal Sinuses

Abstract: This review suggests that an endoscopic approach to EAF tumor is a viable option, frequently yielding complete resection.

Cited by 12 publications

References 40 publications

Eosinophilic angiocentric fibrosis and immunoglobulin 4‐related disease revisited

Eosinophilic angiocentric fibrosis and immunoglobulin 4‐related disease revisited

A rare lumpy inflammatory lesion of the orbit: a unique case report

Eosinophilic Angiocentric Fibrosis of the Nasal Cavities: A Report of an Uncommon Lesion with Emphasis on the Etiology and Differential Diagnosis

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