Merza Nooraldin, Taha Ahmed, Lung John, Benderman Anthony W, Wright Stephen E
Department of Internal Medicine, Texas Tech University Health Sciences Center, Amarillo, TX, USA.
School of Medicine, Texas Tech University Health Sciences Center, Amarillo, TX, USA.
Case Reports Immunol. 2019 Jun 18;2019:3618510. doi: 10.1155/2019/3618510. eCollection 2019.
Immunoglobulin G4-related disease (IgG4-RD) is known for forming soft tissue mass lesions that may have compressive effects. It is an extremely rare disease that most frequently affects the pancreas causing autoimmune pancreatitis. It can also affect the gallbladder, salivary glands, and lacrimal glands causing respective organ-specific complications. In our report, we describe an IgG4-RD case that affected the spinal cord. A 60-year-old female presented with cervical spinal cord compression caused by IgG4-RD leading to several neurological deficits. Pathological examination of the excisional biopsy of the mass revealed dense lymphoplasmacytic cells infiltration and stromal fibrosis with IgG4 and plasma cells. The patient showed a dramatic response to the administration of systemic steroids with almost resolution of her neurological symptoms. This case highlights the first case in literature for IgG4-RD of the extradural tissue causing spinal compression. Hereby, we also demonstrate the dramatic response of IgG4-RD to the administration of systemic steroids as the patient had no recurrence after 5 years of close follow-up, the longest reported period of follow-up reported in the literature to date.
免疫球蛋白G4相关疾病（IgG4-RD）以形成可能具有压迫作用的软组织肿块病变而闻名。它是一种极为罕见的疾病，最常累及胰腺导致自身免疫性胰腺炎。它也可累及胆囊、唾液腺和泪腺，引起各自器官特异性并发症。在我们的报告中，我们描述了一例累及脊髓的IgG4-RD病例。一名60岁女性因IgG4-RD导致颈段脊髓受压，出现多种神经功能缺损。对肿块切除活检的病理检查显示有密集的淋巴细胞和浆细胞浸润以及伴有IgG4和浆细胞的间质纤维化。患者对全身使用类固醇治疗反应显著，神经症状几乎完全缓解。该病例是文献中首例关于硬膜外组织IgG4-RD导致脊髓压迫的病例。在此，我们还展示了IgG4-RD对全身使用类固醇治疗的显著反应，因为该患者在密切随访5年后未复发，这是迄今为止文献报道的最长随访期。
