A Case of Immunoglobulin G4-Related Sclerosing Disease not Responded to Steroid in Nasal Cavity

Ko, Seok Hwa; Yoon, Hyung Joon; Zheng, Tao; Jeong, Jin Hyeok

doi:10.18787/jr.2017.24.2.138

Cited by 3 publications

(1 citation statement)

References 14 publications

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“…[5][6][7][8][9] 본 증례에서는 후비루(SNOT-22 3점)가 주증상으로 나타났으며, 양측 코막힘(SNOT-22 1점), 경미한 후각 저하(SNOT-22 1점) 및 비 건조감(Visual Analogue Scale [VAS] 2점)과 잦은 가피 형성(VAS 2점)의 임상증상을 보였다.IgG4-연관 비부비동 질환은 대개 다른 장기의 침범을 동 반하는 것으로 알려졌으나 비부비동만 단독으로 침범하는 경 우도 있다. 10) 국내에서는 뇌하수체 침범을 동반한 사례가 1예 있고, 그 외 5예는 모두 비부비동 단독 침범 사례이다.…”

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A Case of IgG4-Related Disease With Sino-Nasal Involvement

Han¹,

Sang²,

Jung³

et al. 2022

Korean J Otorhinolaryngol-Head Neck Surg

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IgG4-related diseases are immune-mediated diseases associated with fibroinflammatory lesions and are known to invade almost all organs of the human body. In the head and neck area, it rarely occurs in the nasal cavity and sinuses. Diagnosis is made by combining clinical features, serological, imaging, and histopathological findings, and glucocorticoids are known as the main treatment. In general, surgical resection is not regarded as a curative treatment option. Here we report a case with literature review of IgG4-related diseases with sino-nasal involvement experiencing prominent nasal symptom relief and remission with surgical treatment, which did not respond well to oral steroid in a 70-year-old male patient.

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A Case of IgG4-Related Disease With Sino-Nasal Involvement

Han¹,

Sang²,

Jung³

et al. 2022

Korean J Otorhinolaryngol-Head Neck Surg

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IgG4-related disease presenting as otogenic skull base osteomyelitis

Yim

Ahn

et al. 2021

Auris Nasus Larynx

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IgG4-related disease (IgG4-RD) is an emerging clinical disease entity characterized by tumefactive lesions at multiple sites with a dense lymphoplasmacytic infiltrate rich in IgG4 + plasma cells.Although almost any organ can be affected, IgG4-RD is most likely to involve the submandibular, lacrimal, or parotid glands in the head and neck region. However, skull base involvement presenting as otogenic skull base osteomyelitis (SBO) is rare. We encountered a 70-year-old male with IgG4-RD presenting primarily with severe otalgia and otorrhea. He had uncontrolled diabetes mellitus and showed clinical manifestations of otogenic SBO. Tissue immunostaining revealed typical features of increased IgG4-positive plasma cells, and hematological examination showed elevated serum IgG4 concentrations. Treatment with corticosteroids significantly improved wellbeing and partially resolved the lesion based on computed tomography (CT) scan.

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A Case of IgG4 Related Disease in the Nasal Cavity and Paranasal Sinus : A Rare Cause of Nasal Bleeding

Mun¹,

Shin²,

Koh³

et al. 2019

J Clin Otolaryngol Head Neck Surg

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Immunoglobulin (Ig) G4-related disease is a systemic chronic inflammatory disease characterized by sclerosing lesions by IgG4 positive plasma cells and T lymphocytes. It is rarely described in the sinonasal cavity and the paranasal sinus. Here we report a case involving a 69-year-old woman whose chief complaint was recurrent nasal bleeding for 2 years. Her nasal cavity showed erosion of nasal septum as well as the synechia in between the nasal septum and the lateral nasal wall with severe edematous change in the left inferior turbinate. Imaging finding showed bilateral soft tissue density in the maxillary and ethmoid sinuses and mucosal swelling of left nasal cavity. Her IgG4 level was elevated and proteinase-3 Ab was positive. Histopathological examination from the left inferior turbinate showed dense lymphoplasma cell infiltration and storiform fibrosis. Immunostaining for IgG4 and IgG revealed numerous plasma cells positive for IgG4 (more than 90 cells per high power field) and IgG4/IgG ratio of 0.7. Otorhinolaryngologist should consider IgG4 related disease as one of the differential diagnosis in case of bloody rhinorrhea with mucosal erosion or synechia inside the nasal cavity. (J Clinical Otolaryngol 2019;30:116-121) KEY WORDS：IgG4-related diseaseㆍPlasma cellㆍNasal cavityㆍParanasal sinus

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A Case of Immunoglobulin G4-Related Sclerosing Disease not Responded to Steroid in Nasal Cavity

Cited by 3 publications

References 14 publications

A Case of IgG4-Related Disease With Sino-Nasal Involvement

A Case of IgG4-Related Disease With Sino-Nasal Involvement

IgG4-related disease presenting as otogenic skull base osteomyelitis

A Case of IgG4 Related Disease in the Nasal Cavity and Paranasal Sinus : A Rare Cause of Nasal Bleeding

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