IgG4相关下颌下腺炎伴局部淋巴结病变的临床与病理特点分析

目的 :分析IgG4相关下颌下腺炎伴局部淋巴结病变的临床和病理特点,并深入探讨其鉴别诊断与治疗方法。方法:回顾2007-09—2015-08期间,诊断为IgG4相关下颌下腺炎性病变患者14例,其中男性5例,女性9例,年龄范围16~75岁,分析临床及影像学表现和病理特点。结果:IgG4相关下颌下腺炎可发生于多个年龄段,下颌下腺肿大可伴口干、眼干及泪腺肿大症状,血清IgG4分类水平3 380~21 600mg/L。增强CT表现为弥漫性肿大,均匀强化,边界清晰。病理显示小叶结构破坏,大量IgG4阳性细胞浆细胞浸润伴有淋巴滤泡形成,席纹状纤维化和闭塞性静脉炎。淋巴结病变表现为大量的淋巴滤泡伴增生活跃的生发中心。结论:IgG4相关疾病是一类病因不明的纤维化炎症性疾病,有独特的临床及病理表现。活检确诊IgG4相关下颌下腺炎的患者,建议进行全身系统性评估,发现其他器官累及可能,以利整体治疗计划的制定。     

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。     

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3例报告及文献复习

目的分析探讨IgG4相关唾液腺炎病例的临床特点、诊断及治疗预后,提高对该病的认识。 方法回顾性分析3例IgG4相关唾液腺炎的临床表现、血清学、病理学及治疗效果并进行相关文献复习。 结果（1）病例报告：3例患者主要临床表现为颌下腺肿大,其中双侧颌下腺2例、单侧颌下腺1例。血清学检测显示IgG4、IgG、κ链、λ链、SAA、补体C3、C4等不同程度升高;超声显示腺体弥漫性肿大;病理学检测显示,以淋巴细胞、浆细胞浸润为主,IgG4（+）浆细胞≥30个/HPF,IgG4/IgG≥40%;治疗主要采用糖皮质激素,治疗效果良好未复发。（2）文献复习：国内有关IgG4相关唾液腺炎的报道80例,均表现为单侧或双侧唾液腺无痛性肿胀或肿块,部分患者血清IgG4表达升高,病理学表现为以淋巴细胞、浆细胞浸润为主,IgG4（+）细胞≥50个/HPF,IgG4/IgG≥40%。糖皮质激素及免疫抑制剂联合治疗预后良好。 结论IgG4相关唾液腺炎属自身免疫性疾病,表现为唾液腺无痛性肿大、血清IgG4异常升高,病理特征为组织内大量IgG4（+）浆细胞浸润,临床上怀疑此病时应尽早行活检或血清学检查,确诊后首选糖皮质激素及免疫抑制剂联合治疗的方案,避免不必要的手术及放化疗。     

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。     

累及口腔的IgG4相关疾病研究进展

IgG4相关疾病(immunoglobulin G4-related disease, IgG4-RD)是一种病因不明的全身性多器官纤维炎性疾病,研究认为B细胞和T细胞的增殖活化及其相互作用在该病的免疫反应及纤维化过程中可能发挥重要作用。IgG4-RD可累及包括口腔在内的多个器官和系统。唾液腺是口腔内最常受累的器官,此外,口腔的其他组织也可被IgG4-RD累及,且临床表现异质,临床准确诊断存在困难。为此,该文检索近年来IgG4-RD累及口腔组织的文献报道,结合最新诊断标准,从流行病学、发病机制、临床表现、诊断与治疗出发作一综述。     

慢性硬化性颌下腺炎IgG4的表达观察

目的 分析慢性硬化性颌下腺炎的临床病理特点及IgG4的表达,为临床诊断和治疗提供参考.方法 收集术后病理诊断为慢性硬化性颌下腺炎的病例9例,男性6例,女性3例,年龄51 ～77岁,中位年龄66岁.对其临床资料、手术标本病理组织学特征及免疫表型总结分析.结果 组织学表现为颌下腺小叶结构保存,致密的淋巴细胞和浆细胞浸润,大量淋巴滤泡反应性增生,腺泡不同程度萎缩,小叶间隔明显纤维化,静脉炎及闭塞性静脉炎形成.免疫组织化学检测显示病变组织中IgG4及IgG阳性的浆细胞均明显增加,每一高倍视野内分别平均为186个和261个,IgG4/IgG为0.71.其中3例患有系统性IgG4相关硬化性疾病.结论 慢性硬化性颌下腺炎是一种IgG4相关硬化性疾病,确诊后应排除是否存在涎腺外的IgG4相关硬化性疾病,并密切随访,类固醇激素治疗效果良好.     

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。     

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...     

IgG4相关疾病21例临床分析

目的总结IgG4相关疾病在头颈部的临床表现,探索其治疗方法。方法对诊断为IgG4相关性疾病的患者21例,对患者的临床资料、使用糖皮质激素及免疫抑制剂进行治疗的结果进行回顾性分析。结果 21例确诊患者唾液腺体均可触及肿大质硬包块,CT显示肿大腺体内不规则高密度影像、均匀强化,部分患者伴有口腔、鼻腔、鼻窦、咽喉部等黏膜增厚、肿块样变,多数患者伴有颈部淋巴结肿大,血清IgG4水平升高(≥1.35 g/L)。所有患者头颈部受累唾液腺、泪腺腺体、受累黏膜、淋巴结组织病理学检查均可见淋巴细胞、浆细胞浸润、IgG4阳性浆细胞浸润,21例患者在使用糖皮质激素后所受累腺体内及黏膜处肿块(包括头颈部及其他组织)均消失,临床症状得到缓解,但随着糖皮质激素用量减少后会再次复发、甚至出现加重。结论对于单个颌下腺、腮腺内肿物和伴有其他唾液腺肿大、淋巴结肿大的患者,首选CT鉴别腺体内肿物性质,并结合血液检查、病变部位组织病理学检查,临床上明确诊断为IgG4相关疾病后,使用糖皮质激素治疗以获得良好预后,并需要长期随访,并及时调整用药方案。     

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.     

Differential diagnosis of immunoglobulin G4-related sialadenitis and Kimura’s disease of the salivary gland: a comparative case series

The aim of this study was to investigate key points for the differential diagnosis of immunoglobulin G4-related sialadenitis (IgG4-RS) and Kimura’s disease (KD) involving the salivary glands. The clinical, serological, radiological, histological, and immunohistochemical features of 85 IgG4-RS cases and 52 KD cases were evaluated comparatively. Seventy-two IgG4-RS cases had enlargement of multiple salivary and/or lacrimal glands; 67 patients had bilateral submandibular gland (SMG) involvement. Unilateral parotid gland involvement (59.6%) and comorbid skin lesions (61.5%) were common in KD. Serum IgG4 was elevated in 94.1% of IgG4-RS cases versus 19.0% of KD cases (cut-off value = 266.5 mg/dl). KD was more commonly associated with elevated eosinophil counts (86% vs 23.1%) and elevated IgE concentrations (95.5% vs 76.6%). Storiform fibrosis, irregular lymphoid follicles, and increased IgG4-positive cells (112.9 ± 37.6/high-power field (HPF)) were common in IgG4-RS. Acellular fibrosis, regular lymphoid follicles, IgE-positive reticular networks, increased IgE-positive cells (43.4 ± 26.7/HPF), and tryptase-positive mast cells (29.7 ± 13.3/HPF) were usually detected in KD. Computed tomography showed that 85.7% of KD cases involved subcutaneous fat tissue. A superficial hypoechoic and reticular pattern with multiple hypoechoic foci were the sonographic features of the SMG in IgG4-RS. Despite numerous overlapping manifestations, histopathological examination showed meaningful differences in the types of fibrosis, eosinophils, and IgG4-positive cell counts. Comprehensive evaluation of clinical, serological, radiological, and histopathological features are crucial for the differential 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.     

The diagnostic utility of biopsies from the submandibular and labial salivary glands in IgG4-related dacryoadenitis and sialoadenitis,so-called Mikulicz's disease

IgG4-related dacryoadenitis and sialoadenitis (IgG4-DS) is characterized by serum IgG4 elevation and the infiltration of IgG4-positive plasma cells in glandular tissues. For definitive diagnosis of IgG4-DS, biopsies of local lesions are recommended to exclude Sjögren's syndrome (SS), malignant tumours, and similar disorders. In this study, we examined the diagnostic utility of submandibular gland (SMG) and labial salivary gland (LSG) biopsies in IgG4-DS. Fourteen patients presenting with swelling of the SMG (eight females and six males) underwent both SMG and LSG biopsies. The sensitivity, specificity, and accuracy of SMG biopsies were all 100.0%. In contrast, those of LSG biopsies were 69.2%, 100.0%, and 71.4%, respectively. Thirty-three out of 61 LSG biopsies (54.1%) from all 14 patients were positive for the diagnostic criteria of IgG4-DS (IgG4-positive/IgG-positive plasma cells >0.4). None of the patients experienced complications such as facial nerve palsy, sialocele, or hyposalivation. The IgG4/IgG ratio showed no significant correlation between the LSG and SMG. The final diagnosis was IgG4-DS in 13 patients and marginal zone B-cell lymphoma (MZL) in one. These results suggest that incisional biopsy of the SMG is useful and appropriate for the definitive diagnosis of IgG4-DS, while diagnosis by LSG biopsy alone requires more caution.     

Effect of salivary secretion with mouthguard use on seasonal allergic rhinitis symptom improvement

ObjectivesIt was shown that mucosal immunity via salivary IgA may be related to the improvement of seasonal allergic rhinitis (SAR) symptoms, and improvement of SAR symptoms through saliva flow increase has been reported in patients using mouthguard (MG) in dental treatment. The purpose of this study was to analyze the effect of MG use on SAR symptom improvement and to clarify the role of saliva on SAR symptom development.MethodsWe recruited patients from the Kanagawa Dental University Hospital including 38 and 8 patients with SAR and non-SAR symptoms during two seasons from March 2017 to April 2018. We analyzed the saliva flow rate pre- and post-MG use and measured the amount of IgA and IgG4 in the saliva. We assessed the correlation between SAR symptoms and MG use. SAR symptoms were examined according to a specific clinical score.ResultsIt was revealed that salivary IgA concentration was significantly lower in SAR patients than in controls. SAR symptoms significantly improved with MG use. The saliva flow rate and IgA levels significantly increased with MG use, although the IgG4 levels did not change.ConclusionsMG use may be beneficial for improving the symptoms of SAR patients by increasing the IgA levels.Trial registrationUniversity Hospital Medical Information Network Clinical Trials Registry (UMIN-CTR: UMIN000026428) on 6^thMarch 2017.