IgG4 related disease masquerading as a sinonasal mass: a case report and review of literature


  • Meenu Krishnan Department of Otorhinolaryngology, Manipal Hospitals, Whitefield, Bengaluru, Karnataka, India
  • Vijay Rangachari Department of Otorhinolaryngology, Manipal Hospitals, Whitefield, Bengaluru, Karnataka, India https://orcid.org/0000-0002-0085-9777
  • Sanjeev Katti Department of Histopathology, Manipal Hospitals, Whitefield, Bengaluru, Karnataka, India
  • Yathish G. Channabasappa Department of Rheumatology, Aster Whitefield Hospitals, Bengaluru, Karnataka, India
  • Susmita H. Rakshit Department of Histopathology, Aster Whitefield Hospitals, Bengaluru, Karnataka, India




IgG4- related disease, Sinonasal IgG4, Paranasal IgG4


IgG4-related disease (IgG4-RD) is a relatively new autoimmune fibroinflammatory disease. Most seen to affect the pancreas and biliary tract, IgG4-RD of the nose and paranasal sinuses is very rare. Known to have tumour-like clinical presentations, they can present with varying levels of dysfunction of the organs they affect. In the paranasal sinuses, it can present with nasal block, discharge, bleeds, and sometimes, with symptoms that arise as a consequence of bony erosions and subsequent extension of the disease. We present a case of a 52-year-old lady, who presented with a painful swelling over the malar region with discoloration of the overlying skin. Radiological evaluation helps to check for bony erosions and soft tissue infiltration. Histopathology with immunohistochemistry helps to confirm the diagnosis of IgG4-RD. Most patients benefit from a long course of oral steroids and regular follow-ups, that help to prevent recurrence and ensure a good quality of life for the patient.


Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25(9):1181-92.

Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001;344(10):732-8.

Mulholland GB, Jeffery CC, Satija P, Côté DW. Immunoglobulin G4-related diseases in the head and neck: a systematic review. J Otolaryngol Head Neck Surg. 2015;44(1):24.

Kamisawa T, Zen Y, Pillai S, Stone JH. IgG4-related disease. Lancet. 2015;385(9976):1460-71.

Takano K, Yamamoto M, Takahashi H, Himi T. Recent advances in knowledge regarding the head and neck manifestations of IgG4-related disease. Auris Nasus Larynx. 2017;44(1):7-17.

Døsen LK, Jebsen P, Dingsør B, Haye R. IgG4-Related Nasal Pseudotumor. Case Rep Otolaryngol. 2015;749890.

Kaur K, Kakkar A, Manchanda S, Chatterjee P, Kaur H, Mishra D, et al. Sinonasl Ig-G4 related disease: a rare and emerging entity broadening the differential diagnosis in the sinonasal universe. Eur Arch Otorhinolaryngol. 2021;278(8):2883-90.

Wilson CP, Brownlee BP, El Rassi ET, McKinney KA. Sinonasal immunoglobin G4—related disease: case report and review. Clin Case Rep. 2021;9(12):e05095.