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Sinonasal IgG4-related disease: a rare and emerging entity broadening the differential diagnosis in the sinonasal universe

  • Rhinology
  • Published:
European Archives of Oto-Rhino-Laryngology Aims and scope Submit manuscript

Abstract

Background

IgG4-related disease (IgG4-RD) is a multi-organ immune-mediated disorder characterized by fibroinflammatory mass-forming lesions, mimicking malignancy or infection. While well-documented in salivary glands, orbit and thyroid in the head and neck, sinonasal IgG4-RD is rare.

Methods

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

Results

Seven cases of sinonasal IgG4-RD were identified over a 2-year period, including three males and four females, with an age range of 13–48 years (median: 32 years). Patients presented with cheek swelling, pain and visual disturbances. Serum IgG4 levels were mildly elevated. Storiform fibrosis, obliterative phlebitis and plasma cell infiltration were seen in varying proportions. Destruction of bone and subepithelial mucoserous glands was present. ALK-1 negativity distinguished from inflammatory myofibroblastic tumor.

Conclusion

Sinonasal IgG4-RD expands the growing spectrum of IgG4-RD. A high degree of suspicion is required to include IgG4-RD in differential diagnosis of sinonasal masses, and perform detailed histological and immunohistochemical workup for accurate diagnosis.

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Funding

The authors received no financial support for the research, authorship, and/or publication of this article.

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Authors and Affiliations

  1. Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India

    Kanwalpreet Kaur, Aanchal Kakkar & Deepali Jain

  2. Department of Radiology, All India Institute of Medical Sciences, New Delhi, 110029, India

    Smita Manchanda & Ashu Seith Bhalla

  3. Department of Otorhinolaryngology and Head Neck Surgery, All India Institute of Medical Sciences, New Delhi, 110029, India

    Puja Chatterjee, Hitesh Verma, Rajeev Kumar & Prem Sagar

  4. Department of Oral Pathology and Microbiology, Centre of Dental Education and Research, All India Institute of Medical Sciences, New Delhi, 110029, India

    Harpreet Kaur & Deepika Mishra

Authors
  1. Kanwalpreet Kaur
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  2. Aanchal Kakkar
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  3. Smita Manchanda
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  4. Puja Chatterjee
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  5. Harpreet Kaur
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  6. Deepika Mishra
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  8. Rajeev Kumar
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  9. Prem Sagar
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  10. Deepali Jain
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  11. Ashu Seith Bhalla
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Contributions

KK conceived and designed the study, compiled and interpreted the data, drafted the manuscript. AK conceived and designed the study, supervised the data collection and analysis, had final approval of the manuscript. SM, ASB provided radiological details. PC, HK, DM, HV, RK, PS were treating clinicians of the patients and provided clinical data. DJ, involved in acquisition and analysis of data.

Corresponding author

Correspondence to Aanchal Kakkar.

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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 278, 2883–2890 (2021). https://doi.org/10.1007/s00405-020-06564-w

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  • DOI: https://doi.org/10.1007/s00405-020-06564-w

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