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Abstract Number: 243

Eosinophilic Angiocentric Fibrosis : A Mimic of Vasculitis in IgG4 Related Disease Spectrum

Raphaël Lecomte1, Antoine Néel2, Olivier Malard3, Jérôme Martin4, Michael Hénoux3, Elisabeth Cassagnau5 and Mohamed Hamidou6,7, 1Internal Medicine Department, CHU Nantes, Nantes, France, 2Department of Internal Medicine, Nantes University Hospital, Nantes, France, 3ENT, ENT department, Nantes University Hospital, Nantes, France, 4Immunology laboratory, Immunology laboratory, Nantes University Hospital, Nantes, France, 5Histopathology, Histopathology department, Nantes University Hospital, Nantes, France, 6Hotel Dieu, Service de médecine interne, Hôpital Universitaire de Nantes, Nantes, France, Nantes, France, 7Internal Medicine Department, Internal Medicine Department, Nantes University Hospital, Nantes, France

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: IgG4 Related Disease, Plasmablasts and Wegener's granulomatosis

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Session Information

Date: Sunday, November 13, 2016

Title: Miscellaneous Rheumatic and Inflammatory Diseases - Poster I

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose: Eosinophilic angiocentric fibrosis (EAF) is a rare localized fibro-inflammatory lesion involving usually upper respiratory tract and the orbit. It could mimic ENT manifestations of systemic vasculitis particularly GPA. The diagnosis is based on characteristic histologic findings with a mixed inflammatory infiltrate with eosinophils and fibrosis around small caliber arteries with « onion-skin » pattern. Some autors suggest that EAF is a part of the spectrum of IgG4-related systemic disease.

Methods: We report 3 cases of histology-proven EAF. Demographic, clinical, biological, radiologic and histological features are examined. The 3 patients had a 18-fluorodeoxyglucose positron emission tomography (FDG PET) and evaluation of circulating total plasmablast count (CD19low, CD38+, CD20-, CD27+).

Results: table Two females and one male patients were aged from 29 to 50 years. The disease involved the nasal cavity in two cases and the subglottic area in the third case. The demographic, biological and clinical features (table) were consistent with reports of EAF published previously. All cases demonstrated a dense fibrotic stroma with a perivascular onion skin type pattern and an inflammatory infiltrate with eosinophils, lymphocytes and plasma cells. No vasculitis, granuloma formation or necrosis were identified in any of these cases. All patients had a radiological evaluation with a computed tomography (CT) and a FDG-PET. CT evaluations showed expanding soft tissue masses into the maxillary sinus and anterior nasal cavity in 2 cases and soft tissue thickening in the sub-glotting region of the larynx in one case. There was bone destruction in one case. The FDG-PET showed an enhanced FDG uptake of the EAF lesion. No other abnormal hypermetabolic foci were detect on this exam. The serum IgG4 concentration was normal in the 3 cases (from 23 to 50 mg/dL). The circulating plasmablast count was increased in 2 cases (6728/mL et 2958/mL) and normal in the third patient (508/mL). Two patients received glucosteroids associated in one with colchicin without efficiency. Patients with sinonasal lesion underwent partial surgical excision with relapse in the 2 cases. Patient with the subglottic stenosis was improved by endoscopic dilatation.

Conclusion: EAF is an unrecognized entity mimicking a necrotizing vasculitis limited to upper respiratory tract. FDG PET confirms that EAF is a limited pathology without extra-ENT involvment. The anatomopathology examination by an experienced pathologist is the cornerstone of the diagnosis. Despite some differences, EAF share many characteritics with IgG4 related disease like clinical presentation, similar histologic pattern and increase of plasmablast count.

Patient 1 Patient 2 Patient 3
Sex/Age (years) F/34 M/50 F/29
Manifestation Broad nasal ridge Saddle nose deformity Erosive rhinitis/suglottic rhinitis
Blood cell count Normal Normal Normal
Eosinophils (mL) 240 180 120
CRP (mg/L) <5 3.2 <5
Polyclonal hyperglobulinemia (g/L) Yes/14.6 Yes/15.0 Yes/19.3
ANCA Negative Negative Negative
Antinuclear antibodies Negative >1/2560 without specificity 1/640 without specificity
C3/C4 (g/L) 0.79/0.17 1.13/0.23 1.22/0.19
Serum IgG4 (mg/dL) 23 31 50
Plasmablast count (/mL) 672 508 2956
FDG PET uptake Limited Limited Limited

Disclosure: R. Lecomte, None; A. Néel, None; O. Malard, None; J. Martin, None; M. Hénoux, None; E. Cassagnau, None; M. Hamidou, None.

To cite this abstract in AMA style:

Lecomte R, Néel A, Malard O, Martin J, Hénoux M, Cassagnau E, Hamidou M. Eosinophilic Angiocentric Fibrosis : A Mimic of Vasculitis in IgG4 Related Disease Spectrum [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/eosinophilic-angiocentric-fibrosis-a-mimic-of-vasculitis-in-igg4-related-disease-spectrum/. Accessed .
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