Wong W K, Campbell R, Douglas R
Department of Otolaryngology,North Shore Hospital,Auckland,New Zealand.
J Laryngol Otol. 2014 Jul;128 Suppl 2:S10-5. doi: 10.1017/S0022215114001169.
Immunoglobulin G4 related disease is an inflammatory condition characterised by the presence of fibrotic lesions infiltrated by immunoglobulin G4 positive plasma cells. It can arise from almost any region of the body and it is being increasingly recognised in the head and neck. Regardless of the site of involvement, the histopathological resemblance is remarkable. Dense lymphoplasmacytic infiltration, overabundance of immunoglobulin G4 bearing plasma cells and presence of storiform fibrosis are typical findings.
This paper presents two cases of immunoglobulin G4 related disease in which there was involvement of the orbit, the infraorbital nerve and the infratemporal fossa. Diagnosis was established in both cases by biopsying radiologically abnormal tissue in the infratemporal fossa.
An awareness of this condition is required to establish the diagnosis and initiate appropriate therapy. Glucocorticoids are the mainstay of initial treatment. The effectiveness of B-lymphocyte depletion with rituximab has also been reported. Correct diagnosis may spare patients from unnecessarily radical surgery.
免疫球蛋白G4相关疾病是一种炎症性疾病，其特征为存在由免疫球蛋白G4阳性浆细胞浸润的纤维化病变。它几乎可发生于身体的任何部位，在头颈部的发病率日益增加。无论累及部位如何，组织病理学表现都很相似。密集的淋巴细胞浆细胞浸润、大量携带免疫球蛋白G4的浆细胞以及席纹状纤维化的存在是典型表现。
本文介绍了两例免疫球蛋白G4相关疾病，累及眼眶、眶下神经和颞下窝。两例均通过对颞下窝放射学异常组织进行活检得以确诊。
需要认识到这种疾病以便做出诊断并启动适当治疗。糖皮质激素是初始治疗的主要手段。也有报告称利妥昔单抗清除B淋巴细胞有效。正确诊断可使患者避免不必要的根治性手术。
