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. 2022 Oct 11;7(6):1725-1732.
doi: 10.1002/lio2.944. eCollection 2022 Dec.

Sinonasal IgG4-related sclerosing disease: A rare entity and challenging diagnosis

Andrew O Hess  1 , Brian C Lobo  1 , Marino E Leon  2 , Ernesto M Duarte  2 , Jennifer K Mulligan  1   3 , Jeb M Justice  1
Affiliations

Sinonasal IgG4-related sclerosing disease: A rare entity and challenging diagnosis

Andrew O Hess et al. Laryngoscope Investig Otolaryngol. .

Abstract

Objectives: To describe the rare presentation, imaging and histological findings, and treatments in patients with IgG4-related disease (IgG4-RD) and diagnostic pitfalls and difficulties.

Methods: Cases of sinonasal IgG4-RD were retrieved, and clinicopathological features were reviewed.

Results: Seven cases of sinonasal IgG4-RD were identified over an 11-year period, including four males and three females, with an age range of 19-66 years (median 58 years). Patients presented with symptoms related to the mass effect of the lesions or the destructive nature of the disease including fullness, swelling, obstruction, and pain. Serum IgG and IgG4 levels, IgG/IgG4 ratios, storiform fibrosis, obliterative phlebitis, and plasma cell infiltration were seen in varying proportions. Bony erosion and tissue inflammation were present in some cases.

Conclusion: Sinonasal IgG4-RD is exceedingly rare among other IgG4-RD and varied in its clinical presentation thus posing as a clinically difficult disease to diagnosis. Proper clinical, pathological, and immunohistopathological analysis is required for accurate diagnosis. Such disease should be considered in all cases of similar presentation to those in this study.Level of Evidence: 4.

Keywords: IgG4; IgG4‐related disease; paranasal sinus; sinonasal disease.

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Conflict of interest statement

Dr. Justice is a consultant for Medtronic. Dr. Lobo is a consultant for Medtronic and Acclarent.

Figures

FIGURE 1
FIGURE 1
Hard and soft palate perforation/fistula (A). Right nasal mass and facial soft tissue swelling (arrow) (B)
FIGURE 2
FIGURE 2
Total septal perforation, turbinates, lamina papyracea, and cribriform absent on coronal CT (A). Axial CT of bilateral anterior inferior nasal septal lesion (*) (B). Coronal CT showing right nasolabial pyriform aperture mass (*) (C). Destruction of hard palate and septum with right maxillary sinus mass and infiltration of lacrimal system on coronal CT (D)
FIGURE 3
FIGURE 3
Shows the same area of plasmacytic infiltrate stained with IgG (A) and IgG4 (B) at 20X. An increase in the number of IgG4 positive cells is evident when compared with the positive IgG plasma cells in the same area
FIGURE 4
FIGURE 4
Hematoxylin and eosin (H&E) stain at 10× (A) and 20× (B) show the characteristic storiform fibrosis commonly seen in IgG4‐related disease in association with a lymphoplasmacytic infiltrate
See this image and copyright information in PMC

References

    1. Kaur K, Kakkar A, Manchanda S, et al. Sinonasal IgG4‐related disease: a rare and emerging entity broadening the differential diagnosis in the sinonasal universe. Eur Arch Otorhinolaryngol. 2021;278(8):2883‐2890. - PubMed
    1. Hamano H, Kawa S, Horiuchi A, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001;344(10):732‐738. - PubMed
    1. Kouwenberg WL, Dieleman FJ, Willems SM, Rosenberg AJWP. Inflammatory pseudotumour of the alveolar process of the maxilla as clinical manifestation of IgG4‐related disease: a case report and literature review. Int J Oral Maxillofac Surg. 2020;49(6):722‐725. - PubMed
    1. Cain RB, Colby TV, Balan V, Patel NP, Lal D. Perplexing lesions of the sinonasal cavity and skull base: IgG4‐related and similar inflammatory diseases. Otolaryngol Head Neck Surg. 2014;151(3):496‐502. - PubMed
    1. Wallace ZS, Stone JH. An update on IgG4‐related disease. Curr Opin Rheumatol. 2015;27(1):83‐90. - PubMed

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