IgG4-related disease as multiple head and neck swellings: supported by histopathology and immunohistochemistry.

Romanian journal of ophthalmology Pub Date : 2022-04-01 DOI:10.22336/rjo.2022.32

Obaidur Rehman, Dipankar Das, Kasturi Bhattacharjee, Vatsalya Venkatraman, Harsha Bhattacharjee, Apurba Deka

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Abstract

A 66-year-old North-East Indian male presented with bilateral eyelid swelling, ptosis, and bilateral submandibular gland enlargement. Dry skin on both arms was another peculiar complaint. Contrast enhanced CT scans revealed homogenously enhancing, diffusely enlarged lacrimal glands and blood investigations showed raised serum IgG4 levels. Histopathology from lacrimal gland biopsy showed lymphoplasmacytic infiltrates in storiform pattern. Immunohistochemistry showed 35% plasma cells positive for IgG4. A diagnosis of IgG4-related disease was made, due to supportive histopathology, immunohistochemistry, and serum IgG4 levels. The patient showed excellent response to systemic immunomodulators. Abbreviations: IgG4-RD = IgG4-related disease, CECT = Contrast-enhanced computed tomography, ACE = Angiotensin converting enzyme, IHC = Immunohistochemistry, HPF = high power field, IgG4-ROD = IgG4-related ophthalmic disease, ACR/ EULAR = American College of Rheumatology/ European League Against Rheumatism.

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igg4相关疾病为多发性头颈部肿胀:组织病理学和免疫组织化学支持

66岁印度东北部男性，双侧眼睑肿胀，上睑下垂，双侧下颌腺肿大。双臂皮肤干燥是另一个特别的抱怨。CT增强扫描显示泪腺均匀增强，弥漫性增大，血液检查显示血清IgG4水平升高。泪腺活检病理显示淋巴浆细胞浸润呈故事状。免疫组化显示35%的浆细胞IgG4阳性。根据支持性组织病理学、免疫组织化学和血清IgG4水平，诊断为IgG4相关疾病。患者对全身免疫调节剂表现出良好的反应。缩写:IgG4-RD = igg4相关疾病，CECT =对比增强计算机断层扫描，ACE =血管紧张素转换酶，IHC =免疫组织化学，HPF =高倍视场，IgG4-ROD = igg4相关眼科疾病，ACR/ EULAR =美国风湿病学会/欧洲风湿病联盟。

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Romanian journal of ophthalmology

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