IgG4相关唾液腺炎临床诊疗特点

目的:探讨IgG4相关唾液腺炎的临床特点、诊断和治疗方法。方法:回顾性分析10例IgG4相关唾液腺炎患者临床检查、血清学、病理学和B超检查资料,并观察随访小剂量糖皮质激素疗效。结果:10例IgG4相关唾液腺炎患者均表现为双侧唾液腺持续、对称、无痛性肿大,血清IgG4浓度异常升高,可伴有腺体IgG4+浆细胞浸润合并组织纤维化或硬化;自身抗体均为阴性;B超表现腺体常呈蜂窝状弥漫性病变;小剂量糖皮质激素口服治疗,短期疗效明显。结论:IgG4相关唾液腺炎有其特有的临床表现,应注意与舍格伦综合征(简称SS)和唾液腺肿瘤相鉴别,短期内小剂量糖皮质激素治疗有效。     

IgG4相关唾液腺炎的临床研究进展

<正>IgG4相关疾病是一类新近被认识的与IgG4淋巴浆细胞密切相关的慢性系统性疾病,以病变组织弥漫性肿大、密集的淋巴浆细胞浸润(富含IgG4+浆细胞)、席纹状纤维化为特征,常伴有血清IgG4浓度升高。20世纪60年代人们首次发现自身免疫性胰腺炎(autoimmune pancreatitis,AIP)这一对激素治疗敏感的炎性疾病,直到2001年,Hamano等[1]偶然发现AIP患者常伴有血清IgG4浓度升高,胰腺穿     

IgG4相关唾液腺炎研究现状

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。     

免疫球蛋白G4相关性唾液腺炎临床特点及诊疗进展

免疫球蛋白G4相关性唾液腺炎（IgG4-RS）是一种与IgG4密切相关的慢性自身免疫性疾病,其发病率低,病因和发病机制尚不明确,以唾液腺尤其是颌下腺无痛性持续性肿大为主要临床表现,多数患者血清IgG4升高,淋巴浆细胞浸润,后期全身多器官可继发病变,组织病理学是该病最可靠的诊断方法。临床上倾向于传统糖皮质激素作为首选用药,现阶段可尝试应用生物制剂进行靶向治疗。本文根据国内外文献,对IgG4-RS的病因、临床表现、诊断与鉴别诊断、治疗和预后的研究进展作一综述,以期为该病的临床诊疗和研究提供帮助。     

累及舌体的IgG4相关性疾病1例

IgG4-相关性疾病(IgG4-RD)是一种免疫介导的炎症伴纤维化疾病,但以舌体病变为主要症状的未见报道.现报告1例发生于舌体的IgG4相关性疾病的患者,报道其病例特点及诊治体会.     

超声弹性成像技术在IgG4相关唾液腺炎诊断和随访中的应用

目的: 探讨超声弹性成像技术在IgG4相关唾液腺炎诊断和随访中的应用价值。方法: 回顾分析2018年5月—10月就诊于上海交通大学医学院附属第九人民医院口腔外科,诊断为IgG4相关唾液腺炎3例患者的临床资料。所有患者均接受口服小剂量糖皮质激素治疗,采用超声弹性成像技术应变率比值法和声辐射力脉冲成像法,对治疗前、后患者下颌下腺进行弹性超声检查,观察超声影像学特点及疗效。结果: 3例患者临床表现均为双侧下颌下腺无痛性肿大超过3个月,血清IgG和IgG4浓度均异常升高,下颌下腺活检病理及免疫组织化学染色结果提示符合IgG4相关唾液腺炎。治疗前弹性超声成像显示腺内大范围蓝色区域对应低回声区,应变率比值（SR）较正常腺体减小而剪切波速值（SWV）较正常腺体增大,表示该区域腺体质地比正常腺体明显较硬。所有患者经小剂量糖皮质激素治疗1个月后随访,临床表现原本肿大的下颌下腺明显消退,触诊质地软化,血清IgG和IgG4浓度明显下降,弹性超声显示蓝色区域范围明显缩小,黄色和绿色区域范围增大,对应SR增大而SWV减小,客观且半量化地反映腺体质地较治疗前软化。结论: 超声弹性成像技术通过测量SR或SWV,可以客观半量化地反映病变唾液腺质地。结合患者的临床表现、血清学指标和组织病理学表现,可以辅助IgG4相关唾液腺炎的诊断,并初步评价疾病的严重性和进展程度。     

IgG4相关硬化性腮腺炎1例报告及文献复习

通过对1例IgG4相关硬化性腮腺炎疾病的诊治的介绍和相关文献的回顾复习,增加国内口腔科医师对该类疾病的认识,以减少临床误诊误治.     

双侧IgG4相关慢性硬化性下颌下腺炎：1例报告及文献复习

IgG4相关性疾病作为一种系统性疾病,可累及多个器官和组织。发生于口腔颌面部的IgG4相关性疾病主要包括Kiittner瘤和Mikulicz病,该病对激素的治疗反应良好,但由于该病少见,临床常常将其误诊为恶性肿瘤而导致不必要的创伤性手术治疗。本文报告l例累及双侧下颌下腺的Kiittner瘤,并结合文献对其发病机制、临床表现、诊断和治疗进行讨论。     

31例IgG4相关性疾病的临床分析

目的:提高对IgG4相关性疾病(IgG4-related disease,IgG4-RD)的认识及诊治水平.方法:分析31例就诊于新疆医科大学第一附属医院诊断为IgG4-RD患者的临床表现、影像学、血清学、病理学、治疗等特点,并使用IgG4-RD应答者指数评估疾病活动和损害.结果:31例患者中,男18例,女13例,平均...     

Inflammatory pseudotumour of the alveolar process of the maxilla as clinical manifestation of IgG4-related disease: a case report and literature review

IgG4-related disease (IgG4-RD) is an uncommon immune-mediated condition considered to be a systemic disease, described in multiple organ systems. IgG4-RD that involves the maxillary and sinonasal region is rare. This report presents a very rare presentation of IgG4-RD in the maxillary alveolar process. The patient presented with left-sided facial pain, headache, and mobility and loss of teeth. The first biopsy and resection specimen reports were inconclusive and showed a non-specific chronic inflammatory process. After the third resection, the diagnosis was finally established through findings that satisfied the 2012 consensus criteria for IgG4-RD. Consequently high doses of oral corticosteroids and azathioprine were given, tapered over a total period of 36 months. Weaning is still in progress, but no recurrence was observed after 34 months. A review of the English-language literature was performed, which identified seven cases of IgG4-RD with maxillary and sinonasal involvement. Cases were excluded from the review if there was any doubt that they met the consensus statement on the pathology.     

超声检查在IgG4相关唾液腺病变诊断中的应用

目的： 探讨IgG4相关唾液腺病变的超声声像图表现,以提高超声科医师对本病的认识。方法：经临床血清学检验及组织病理确诊（手术10例,超声引导下穿刺活检15例）,对25例IgG4相关唾液腺病变的超声声像图进行回顾性分析,包括病变腺体的形态、边界,内部回声,血流分布,腺管有无扩张,腺管内有无结石,邻近有无淋巴结肿大。对比分析14例甾体类药物治疗3个月后的超声声像图改变。结果：25例IgG4相关唾液腺病变患者平均年龄64.5岁,男女比例4∶1。病变位于下颌下腺（20例）、腮腺（5例）。超声表现：① 弥漫性改变10例（9例双侧,1例单侧）,形态饱满,腺体回声偏低,呈网格状改变,3例腺管扩张,2例伴腺管内结石,彩色多普勒显示唾液腺腺体内血管增多,血流丰富;② 局灶性改变15例（14例单侧,1例双侧）,呈不均质偏低回声“肿块”,“肿块”内血流呈放射状分布。③下颌下淋巴结病变8例,4例经穿刺确诊,4例疑似。④ 甾体类药物治疗后,肿大的唾液腺逐渐恢复,局灶性病灶逐渐变小,病变内血流分布减少。结论：认识IgG4相关唾液腺病变的超声图像表现,可以有效辅助临床诊断与治疗后随访,避免不必要的手术风险。     

Utility of navigation system-guided submandibular gland core needle biopsy in the diagnosis of immunoglobulin G4-related sialadenitis

Pathological diagnosis is important for the definite diagnosis of immunoglobulin G4-related sialadenitis (IgG4-RS). Core needle biopsy (CNB) is a scarless technique; however the pathological heterogeneity of IgG4-RS (a particular feature of this disease) could be the potential cause of the inferior diagnostic capability of submandibular gland CNB (SMG-CNB) for IgG4-RS. The aim of this study was to explore technical improvements in SMG-CNB and improve its diagnostic power in IgG4-RS diagnosis. Eighteen patients clinically suspected for IgG4-RS were enrolled and underwent both SMG-CNB and SMG surgical biopsy. A navigation system (Brainlab) was employed during SMG-CNB to obtain representative samples and avoid blood vessel injury. Histopathological and immunopathological findings for the SMG-CNB samples were in good concordance with SMG surgical biopsy. There was no statistically significant difference between SMG-CNB and SMG surgical biopsy in IgG-positive cell count (132.4 ± 59.3 vs 132.2 ± 47.5, P = 0.99), IgG4-positive cell count (102.2 ± 39.7 vs 97.2 ± 27.6, P = 0.67), or IgG4-positive/IgG-positive cell count ratio (78.6% ± 0.1% vs 75.2% ± 0.1%, P = 0.29). A moderate or strong significant correlation was found between SMG-CNB and SMG surgical biopsy for these cell counts and ratio (all P < 0.01). The diagnostic consistency of SMG-CNB and SMG surgical biopsy was 100%. The Brainlab navigation system may assist in collecting representative SMG-CNB samples from typical pathological lesions. Tissues obtained from SMG-CNB are sufficient for the pathological diagnosis of IgG4-RS. Standardized SMG-CNB is expected to replace SMG surgical biopsy for IgG4-RS diagnosis.     

IgG4‐related sialadenitis and Sjögren's syndrome

IgG4‐related disease (IgG4‐RD) has emerged as a new entity in the last decade. It comprises numerous conditions previously thought to be unrelated. Macroscopically, these diseases cause diffuse organ swelling and formation of pseudotumorous masses. Histopathologically, they are characterized by a lymphoplasmacytic infiltrate with increased IgG4+ plasma cells and storiform fibrosis. Despite rapid progress within the last years, our knowledge on these conditions is still fragmented. To date, more than forty organs have been reported to be included in IgG4‐RD, and salivary gland involvement is amongst the most common organs affected [IgG4‐related sialadenitis (IgG4‐RS)]. Interestingly, IgG4‐RS shares commonalities with Sjögren's syndrome (SS), like glandular enlargement, sicca symptoms, arthralgias, hypergammaglobulinemia, hypocomplementemia, and circulating antinuclear antibodies. Nonetheless, they differ in that the incidence of anti‐Ro and anti‐La reactivity is not frequently found in patients with IgG4‐RS, their salivary glands are infiltrated by a large number of IgG4+ plasma cells and IgG4‐RS symptoms respond promptly to steroids. The aim of this review was to describe the clinical, serological, histopathological and pathophysiological aspects of IgG4‐RS in the context of IgG4‐RD and highlight the differences between IgG4‐RS and SS.