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IgG4 相关疾病导致的多部位头颈部肿胀：组织病理学和免疫组织化学检查结果支持这一诊断。

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

机构信息

Department of Ocular Pathology, Sri Sankaradeva Nethralaya, Guwahati, Assam, India.

Department of Oculoplasty and Oncology, Sri Sankaradeva Nethralaya, Guwahati, Assam, India.

出版信息

Rom J Ophthalmol. 2022 Apr-Jun;66(2):164-167. doi: 10.22336/rjo.2022.32.

DOI:10.22336/rjo.2022.32
PMID:35935075
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9289777/
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. 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.

摘要

一位 66 岁的东北印度男性出现双侧眼睑肿胀、上睑下垂和双侧颌下腺肿大。双臂干燥的皮肤是另一个特殊的症状。增强对比 CT 扫描显示泪腺均匀强化，弥漫性增大，血液检查显示血清 IgG4 水平升高。泪腺活检的组织病理学显示淋巴浆细胞呈巢状浸润。免疫组化显示 35%的浆细胞 IgG4 阳性。由于支持性的组织病理学、免疫组化和血清 IgG4 水平，诊断为 IgG4 相关疾病。该患者对全身免疫调节剂反应良好。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8cf7/9289777/fd8ed093eab6/RomJOphthalmol-66-164-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8cf7/9289777/1503b6fc2490/RomJOphthalmol-66-164-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8cf7/9289777/6cf50730069d/RomJOphthalmol-66-164-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8cf7/9289777/1ab1b7417aa5/RomJOphthalmol-66-164-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8cf7/9289777/fd8ed093eab6/RomJOphthalmol-66-164-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8cf7/9289777/1503b6fc2490/RomJOphthalmol-66-164-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8cf7/9289777/6cf50730069d/RomJOphthalmol-66-164-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8cf7/9289777/1ab1b7417aa5/RomJOphthalmol-66-164-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8cf7/9289777/fd8ed093eab6/RomJOphthalmol-66-164-g004.jpg

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Medicine (Baltimore). 2017 Mar;96(10):e6205. doi: 10.1097/MD.0000000000006205.
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