Eosinophilic angiocentric fibrosis and immunoglobulin 4‐related disease revisited

Abstract

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.

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

“…EAF is believed to be a manifestation of IgG4-related disease (IgG4-RD) [ 3 ]. Chew et al showed that about 30% of EAF meets the IgG4-RD standard of the 2019 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification scheme [ 4 ]. Of the 18 cases of EAF we reviewed, 3 had increased serum IgG4 levels, 5 had normal serum IgG4 levels, and serum IgG4 levels in the remaining 10 were unknown.…”

“…Eosinophilic angiocentric fibrosis (EAF) is a rare and slow progressive inflammatory disease. The main sites of involvement are the nasal cavity and sinuses, although rare cases can also occur in the orbit, upper respiratory tract, gingiva, and brain [ 1 – 4 ]. The etiology and pathogenesis of this disease are still unclear, and its clinical manifestations are nonspecific and diverse, making it easy to confuse with orbital inflammatory lesions or neoplastic lesions in diagnosis.…”

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

“…EAF is an indolent lesion, sometimes locally destructive and with a predilection for the sinonasal tract. Its etiology is still unclear; it was initially attributed to trauma, hypersensitivity, and/or surgical manipulation, while some authors recently suggested including EAF within the spectrum of IgG4-RSD [ 1 , 14 , 15 ].…”

“…Recently, an attempt to evaluate the relationship between EAF and IgG4-RSD has been made by reviewing literature and using a validated set of criteria [ 15 ]. The result was that a small number of EAF patients met the 2019 ACR/EULAR classification criteria for the diagnosis of IgG4-RSD.…”

“…Since then, an elevated serum IgG4 concentration (>135 mg/dL) has often been reported [ 3 ] in patients with EAF, in addition to immunohistochemical reactivity for IgG4. A recent systematic review of the literature demonstrated how the relationship between EAF and IgG4-RSD still has to be proven [ 15 ] because not all EAF cases are strictly related to IgG4.…”