摘要
IgG4相关唾液腺炎(IgG4-related sialadenitis,IgG4-RS)是近年来才被认识的一类自身免疫性疾病,发病机制尚不明确。IgG4-RS主要累及下颌下腺和腮腺,特征性表现为双侧唾液腺或(和)泪腺的弥漫性无痛性肿大,持续时间一般在3个月以上。部分患者伴有听力下降或听力障碍、鼻窦炎、淋巴结肿大等症状;近一半患者存在不同程度的唾液腺分泌障碍。大多数患者血清IgG4浓度升高,但不能将其做为诊断的唯一标记物。组织病理学仍是诊断的"金标准",目前多采用下颌下腺活检来诊断。组织病理学显示淋巴浆细胞浸润,闭塞性静脉炎,席纹状纤维化;免疫组化显示IgG4^+/IgG^+浆细胞>40%,且IgG4^+浆细胞/高倍镜视野>10。糖皮质激素被视为治疗该疾病的一线药物,临床上常与环磷酰胺为代表的免疫抑制剂联合用药,但尚没有形成标准规范的用药方案。大部分患者短期治疗效果显著,长期预后仍需进一步研究,对有复发倾向的患者要及时调整激素剂量。未来仍需对该病的发病机制及治疗方案进一步研究,以提高临床诊断率及治疗效果。
IgG4-related sialadenitis(IgG4-RS) is a type of autoimmune disease that has been recognized in recent years,and the pathogenesis remains unclear.IgG4-RS mainly affects the submandibular gland and parotid gland and is characterized by diffuse painless swelling of the bilateral salivary glands and/or lacrimal glands,usually lasting more than 3 months.Some patients have accompanying hearing loss or hearing impairment,sinusitis,lymphadenopathy and other symptoms;nearly half of patients have different degrees of salivary gland secretion disorders.Most patients have elevated serum IgG4 levels,but they cannot be used as the only marker for diagnosis.Histopathology remains the"gold standard"for diagnosis.Presently,submandibular gland biopsy is often used for diagnosis.Histopathology showed lymphoplasmacytic infiltration,occlusive phlebitis,striated fibrosis;immunohistochemistry showed IgG4^+/IgG^+ plasma cells >40%,and IgG4^+ plasma cell/high-power field vision > 10.Glucocorticoids are regarded as first-line drugs for the treatment of this disease.Clinically,glucocorticoids are often combined with immunosuppressive agents such as cyclophosphamide,but no standard drug regimen exists.Most patients have a significant short-term treatment effect,and the longterm prognosis requires further study.Patients with a recurrence tendency should adjust the hormone dose over time.In the future,further research is needed regarding the pathogenesis and treatment of the disease to improve the clinical diagnosis rate and therapeutic effect.
作者
苏伟喆
范亚伟
孟兵
SU Weizhe;FAN Yawei;MENG Bing(Shanxi Medical University School and Hospital of Stomatology,Taiyuan 030001,China;Department of Stomatology,The First Hospi⁃tal of Shanxi Medical University,Taiyuan 030001,China)
出处
《口腔疾病防治》
2020年第1期56-60,共5页
Journal of Prevention and Treatment for Stomatological Diseases
