涉及颅底特殊区域的igg4相关疾病:我们的经验

Sharmistha Chakravarty, Kritika Bhardwaj
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引用次数: 0

摘要

背景:免疫球蛋白g4相关疾病(IgG4- rd)是一种罕见的全身性纤维炎症性疾病,其特征是淋巴浆细胞浸润IgG4阳性浆细胞、层状纤维化和血清IgG4水平升高。它可以影响多个器官系统,经常模仿恶性肿瘤或感染。颅底累及IgG4-RD并不常见,这给诊断和治疗带来了挑战。我们提出了三种地形上不同的IgG4-RD颅底受累模式。病例介绍:本病例系列描述了三例影响不同颅底区域的IgG4-RD患者。第一例患者为一名57岁女性,主诉为头痛,右眼视力在过去两个月突然恶化,伴有上睑下垂和鞍鼻畸形。影像学显示右眼眶尖囊腔内一非均匀多室囊性病变,伴筛窦及蝶窦息肉样粘膜增厚。后筛和蝶骨肿块活检证实IgG4-RD。患者经皮质类固醇治疗后病情好转。第二例患者为50岁男性,右耳疼痛,听力下降,声音嘶哑,面神经麻痹3个月。影像学表现为不均匀、强化的软组织病变,累及右侧腮腺、颈动脉、咀嚼肌、咽旁及咽粘膜间隙,伴少量溶解性病变,累及右侧颞骨岩质部伴邻近硬化。鼻咽活检证实IgG4-RD和皮质类固醇治疗导致临床改善。我们的第三个病例是一个60岁的男性,患有IgG4-RD o鼻咽,鼻塞和分泌物3个月。影像显示鼻咽部、双侧罗森穆勒窝软组织增厚界限不清,疾病向斜坡扩散。鼻咽活检证实了诊断,患者开始口服皮质类固醇,最初有所改善,但病情进展,导致多器官衰竭和死亡。结论:这些病例突出了IgG4-RD累及颅底的不同临床表现和结局。通过组织病理学和皮质类固醇治疗的早期诊断是改善预后的关键。然而,难治性病例可能需要免疫抑制治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
IgG4-related disease involving distinctive areas of skull base: our experience.

Background: Immunoglobulin G4-related disease (IgG4-RD) is a rare, systemic fibro-inflammatory condition characterized by lymphoplasmacytic infiltration with IgG4-positive plasma cells, storiform fibrosis, and elevated serum IgG4 levels. It can affect multiple organ systems, often mimicking malignancies or infections. Skull base involvement in IgG4-RD is uncommon and presents diagnostic and therapeutic challenges. We present three topographically distinct areas of skull base involvement patterns in IgG4-RD.

Case presentation: This case series describes three patients with IgG4-RD affecting distinct skull base regions. The first case involves a 57-year-old female presenting with chief complaint of headache, sudden deterioration of vision in the right eye over the past two months, with ptosis and saddle nose deformity. Imaging revealed a heterogeneous multiloculated cystic lesion in intraconal compartment of right orbital apex with polypoidal mucosal thickening in ethmoid and sphenoid sinus. Biopsy done from posterior ethmoid and sphenoid mass confirmed IgG4-RD. The patient improved with corticosteroid therapy. The second case is a 50-year-old male with right-sided ear pain, decreased hearing, hoarseness of voice and facial nerve palsy for 3 months. Imaging showed a heterogeneous, enhancing soft tissue lesion involving right parotid, carotid, masticator, parapharyngeal and pharyngeal mucosal space with few lytic lesions involving petrous part of right temporal bone with adjacent sclerosis. Biopsy from nasopharynx confirmed IgG4-RD and corticosteroid therapy led to clinical improvement. Our third case is a 60-year-old male with IgG4-RD Oof nasopharynx, nasal obstruction and discharge for 3 months. Imaging revealed an ill defined soft tissue thickening in nasopharynx, bilateral Fossa of Rosenmuller and disease spreading to clivus. Biopsy from nasopharynx confirmed the diagnosis and patient started on oral corticosteroids, initially improved but the disease progressed, leading to multiorgan failure and death.

Conclusion: These cases highlight the diverse clinical presentations and outcomes of IgG4-RD involving the skull base. Early diagnosis through histopathology and initiation of corticosteroid therapy is critical for improving outcomes. However, refractory cases may require immunosupressive therapies.

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