Immunoglobulin G4 (IgG4)-related disease

  • 文章类型: Journal Article
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  • 文章类型: Case Reports
    免疫球蛋白G4(IgG4)相关疾病是一种全身性疾病,其特征是硬化性病变和血清IgG4水平升高。这种情况可以涉及任何器官,但IgG4相关性脊髓性硬脑膜炎相对罕见.在目前的研究中,我们报告一例IgG4相关性脊髓性脑膜炎引起脊髓压迫的病例.一名39岁的男子向我们展示了15天的背痛史和3天的戒断史,下肢无力加重2天。颈部磁共振成像(MRI)显示,在C5-T4水平,沿脊髓的前后边界呈条状异常信号。脑脊液中IgG水平为718.0mg/L。胸部MRI显示带状异常信号,在T1-T6水平沿硬膜囊的前后边界显着增强。组织病理学检查证实IgG4相关性脊髓性脑膜炎。症状迅速恶化,并手术切除椎管内占位病变进行脊柱减压。进行皮质类固醇治疗,患者的运动功能得到轻度改善。IgG4相关疾病可表现为脊髓性脑膜炎并引起脊髓压迫。临床医生应该意识到这种罕见的情况,早期诊断,及时手术减压,应强调适当的皮质类固醇治疗。
    Immunoglobulin G4 (IgG4)-related disease is a systemic disease characterized by sclerosing lesions and an increased serum IgG4 level. This condition can involve any organ, but IgG4-related spinal pachymeningitis is relatively rare. In the current study, we report a case of spinal cord compression caused by IgG4-related spinal pachymeningitis. A 39-year-old man presented to us with a 15-day history of back pain and a 3-day history of dysuresia, exacerbated by weakness in the lower extremities for 2 days. Cervical magnetic resonance imaging (MRI) showed strip-shaped abnormal signals along the anterior and posterior borders of the spinal cord at the C5-T4 levels. The IgG level in cerebrospinal fluid was 718.0 mg/L. Thoracic MRI revealed strip-shaped abnormal signals with remarkable enhancement along the anterior and posterior borders of the dural sac at the T1-T6 levels. Histopathological examination confirmed IgG4-related spinal pachymeningitis. The symptoms worsened rapidly, and surgical resection of the space-occupying lesion in the vertebral canal was performed for spinal decompression. Corticosteroid therapy was administered, and the patient\'s motor functions were mildly improved. IgG4-related disease can manifest as spinal pachymeningitis and cause spinal cord compression. Clinicians should be aware of this rare condition, and early diagnosis, timely surgical decompression, and appropriate corticosteroid therapy should be highlighted.
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  • 文章类型: Case Reports
    UNASSIGNED: To describe a case of immunoglobulin G4 (IgG4)-related dacyroadenitis presenting as bilateral chorioretinal folds from eyeball compression by massively enlarged lacrimal glands.
    UNASSIGNED: A 51-year-old woman with severely enlarged bilateral lacrimal glands was diagnosed as having IgG4-related dacryoadenitis. The glands strongly compressed the globes, forming chorioretinal folds resembling those found in orbital malignancy. Eventual treatment with oral prednisolone dramatically reduced the volume of the lacrimal glands and released globe compression on magnetic resonance imaging. However, the chorioretinal folds remained in the right fundus and symptoms of blurred vision improved but persisted.
    UNASSIGNED: This is the first account of chorioretinal fold formation by severely enlarged lacrimal glands appearing in IgG4-related dacryoadenitis. Chorioretinal fold formation by an enlarged lacrimal gland occurring bilaterally may represent a basis for suspecting IgG4-related dacryoadenitis. Prompt treatment is recommended for patients presenting with very large lacrimal glands to avoid visual impairment.
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