IgG4相关性涎腺炎的临床病理特点

IgG4相关性疾病作为一种全身系统性疾,可累及多个器官及组织.发生于涎腺的IgG4相关性涎腺炎容易与干燥综合征、慢性非特异性涎腺炎、淋巴瘤等多种疾病混淆,延误治疗并给患者带来诸多不必要的手术负担.因此有必要提高对IgG4相关性涎腺炎的认识.本文就IgG4相关性涎腺炎的临床及病理组织学等特点进行综述.     

IgG4相关性疾病的临床病理特征

目的 探讨IgG4相关性疾病(IgG4-related disease,IgG4-RD)的临床病理学特征、诊断及鉴别诊断.方法 回顾性分析10例IgG4-RD患者的临床病理资料,采用免疫组化EnVision两步法检测IgG、IgG4、CD138、Κappa轻链、Lamda轻链抗体在IgG4-RD中的表达.结果 10例I...     

泪腺IgG4相关性眼病19例临床病理分析

目的 探讨泪腺IgG4相关眼病(IgG4-related ophthal-mic disease,IgG4-ROD)的临床病理特征.方法 收集陕西省西安市人民医院(西安市第四医院)2010年12月～2020年12月确诊的19例IgG4-ROD,并收集10例泪腺炎性假瘤和10例泪腺Mikuliczs病作为非IgG4-RO...     

IgG4相关性桥本甲状腺炎的研究进展

IgG4相关性疾病是一种病因不明的全身系统性自身免疫疾病,可累及多个器官及组织,其成为近10年来的研究热点之一。 IgG4相关性桥本甲状腺炎有其独特的临床病理学特点：血清FT4降低,TPOAb、IgG4升高,组织中大量IgG4阳性浆细胞浸润、明显的基质纤维化倾向和甲状腺滤泡上皮的重度变性。 IgG4相关性桥本甲状腺炎是桥本甲状腺炎的一种亚型,甲状腺素治疗效果较好。对于更易发生甲状腺功能减退、甲状腺损伤更明显的IgG4 HT患者可尝试早期应用皮质激素抑制免疫损伤。该文就IgG4相关性桥本甲状腺炎的临床及病理组织学等特点作一综述。     

IgG4相关性疾病临床病理学特征分析

目的：探讨IgG4相关性疾病的病理学形态、免疫表型特征。方法观察12例IgG4相关性疾病的镜下特点,结合免疫组化EnVision两步法染色检测IgG、IgG4、CD138、CD34的表达,分析IgG4相关性疾病的病理学形态特征。结果 IgG4相关性疾病主要表现为组织弥漫性纤维化;伴大量淋巴细胞、浆细胞浸润,围绕血管神经分布;闭塞性静脉炎形成,免疫组化EnVision两步法染色IgG4阳性浆细胞与IgG阳性浆细胞比例>40%。结论 IgG4相关性疾病临床特点和影像学无特异性,易误诊为肿瘤,术前血清IgG4检测可作为疑似病例的首选方法。     

成人IgG4相关性颌下腺疾病的超声表现

目的 分析及归纳IgG4相关性颌下腺疾病的超声影像特征.方法 回顾性分析19例22灶IgG4相关性颌下腺疾病的超声表现,包括颌下腺尺寸、形态、病变范围、内部回声、血流、周围淋巴结.结果 19例22灶IgG4相关性颌下腺疾病的超声表现可分为局限性结节样改变、弥漫性结节样改变、类肿瘤样改变3种类型.局限性结节样改变(2例2灶),病侧腺体的浅侧、被膜下显示不均匀增粗的低回声区,内见条状强回声,血流偏多.弥漫性结节样改变(11例12灶),病侧腺体呈网格状或蜂窝状,具体表现为腺体回声增强、增粗、不均匀、血流丰富,内见弥漫、散在、大小不一的结节样低回声,其周围显示条状强回声.类肿瘤样改变(7例8灶),病侧腺体表现为回声不均匀减低、增粗,内夹杂多发条状强回声,并显示放射状丰富血流.结论 超声检查可作为IgG4相关性颌下腺疾病首选的影像学检查方法.该病超声影像呈局限性结节样改变、弥漫性结节样改变、类肿瘤样改变3种类型.当怀疑患IgG4相关性颌下腺疾病时,应进一步检查IgG4相关性疾病常累及的组织和器官,同时进行超声引导下穿刺活检明确诊断.     

IgG4相关性疾病的研究进展

IgG4相关性疾病是一个新的独立疾病,临床表现复杂多样,可以浸润多种器官,出现器官弥漫性肿胀或局部肿块形成,病理可见IgG4+浆细胞为主的淋巴浆细胞浸润、闭塞性静脉炎、组织席纹状纤维化改变,血清IgG4浓度升高.此病多见于老年男性,对类固醇激素反应敏感.临床常见的受累器官有:浸润唾液腺、泪腺的米库利兹病,浸润下颌下腺的Küttner肿瘤,Riedel's甲状腺炎,腹膜后纤维化,自身免疫性胰腺炎1型等全身多系统疾病.本文从概念,流行病学概况、发病机制,病理特征,临床表现,诊断和治疗作一综述.     

IgG4相关硬化性疾病头颈部病变的研究进展

IgG4相关硬化性疾病(IgG4-related sclerosing disease,IgG4-SD)是新近认识的一种不同于一般慢性炎症和其他自身免疫性疾病的独立临床病理实体.该疾病主要累及胰腺,也可与胰腺外多个器官病变并存,头颈部IgG4-SD主要表现为颌下腺(Kuttner瘤)、泪腺和腮腺(Mikulicz病)以及脑下垂体(淋巴浆细胞性垂体炎)疾病等.目前头颈部IgG4-SD的诊断以及鉴别诊断已取得一定的研究进展.     

IgG4相关性甲状腺疾病研究的最新进展

免疫球蛋白相关性疾病(immunoglobulin G4- related diseases,IgG4- RD)可累及全身各个器官,累及甲状腺者称为IgG4相关性甲状腺疾病(IgG4- related thyroid disease,IgG4- RTD)。其临床特点为受累器官弥漫性或局灶性肿大、硬化,血清IgG4升高。部分IgG4- RD可导致器官衰竭,特别是胰腺、肝脏和胆道、肾脏、甲状腺、肺和主动脉等。IgG4- RTD多表现为甲状腺局部结节或弥漫性肿大,多伴有血清IgG4升高。IgG4- RTD目前病因未明,多认为与遗传因素、病毒感染、抗原抗体反应有关,治疗上绝大多数对糖皮质激素治疗有效。本文概述了近年来IgG4相关性甲状腺疾病的研究进展。     

IgG4-related prostatitis progressed from localized IgG4-related lymphadenopathy

Immunoglobulin G4-related disease (IgG4-RD) is a recently described inflammatory disease involving multiple organs. Prostate involvement with IgG4-RD is very rare. In this report, we describe a case of IgG4-related prostatitis progressed from localized IgG4-related lymphadenopathy. This patient was present with urine retention symptoms. MRI and CT examination revealed the prostatic enlargement and the multiple lymphadenopathy. Serum IgG4 levels were elevated. Prostatic tissue samples resected both this time and less than 1 year earlier showed the same histological type of prostatitis with histopathologic and immunohistochemical findings characteristic of IgG4-RD. The right submandibular lymph nodes excised 2 years earlier were eventually proven to be follicular hyperplasia-type IgG4-related lymphadenopathy. This is the first case of IgG4-RD that began as localized IgG4-related lymphadenopathy and progressed into a systemic disease involving prostate and multiple lymph nodes. This patient showed a good response to steroid therapy. This leads us to advocate a novel pathogenesis of prostatitis, and a novel therapeutic approach against prostatitis. Pathologists and urologists should consider this disease entity in the patients with elevated serum IgG4 levels and the symptoms of prostatic hyperplasia to avoid ineffective medical or unnecessary surgical treatment.     

Ocular adnexal IgG4-related disease has uniform clinicopathology

IgG4-related disease is a recently proposed clinical entity with several unique clinicopathological features. Ocular adnexal IgG4-related disease, however, has not well been clarified. The purpose of the present study was to examine 21 patients (10 men, 11 women; age range, 39–86 years) with ocular adnexal IgG4-related disease. In 17 out of 21 patients (81%), the lacrimal glands were involved and bilateral lacrimal gland swelling was frequently observed ( n  = 12; 70.6%). In contrast, the conjunctiva was not involved in any of the patient. Histology was uniform with marked lymphoplasmacytic infiltration admixed with dense fibrosis, similar to previous reports of IgG4-related disease. Immunostaining detected numerous aggregates of IgG4-positive plasma cells. Serum IgG4 was higher than normal in 10 of the 13 patients tested, although it was measured after treatment in almost all cases. Interestingly, immunoglobulin heavy chain gene rearrangement was detected in two of 17 patients (12%) examined. The present results show that ocular adnexal IgG4-related disease has uniform clinicopathology: that is, disease involving the bilateral lacrimal glands with lymphoid hyperplasia and fibrosis, but not the conjunctiva. And presence of immunoglobulin heavy chain gene rearrangement suggests the possibility of B-cell lymphoma arising in a background of IgG4-related chronic inflammation.     

Immunology of IgG4-related disease

Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition that derives its name from the characteristic finding of abundant IgG4⁺ plasma cells in affected tissues, as well as the presence of elevated serum IgG4 concentrations in many patients. In contrast to fibrotic disorders, such as systemic sclerosis or idiopathic pulmonary fibrosis in which the tissues fibrosis has remained largely intractable to treatment, many IgG4-RD patients appear to have a condition in which the collagen deposition is reversible. The mechanisms underlying this peculiar feature remain unknown, but the remarkable efficacy of B cell depletion in these patients supports an important pathogenic role of B cell/T cell collaboration. In particular, aberrant T helper type 2 (Th2)/regulatory T cells sustained by putative autoreactive B cells have been proposed to drive collagen deposition through the production of profibrotic cytokines, but definitive demonstrations of this hypothesis are lacking. Indeed, a number of unsolved questions need to be addressed in order to fully understand the pathogenesis of IgG4-RD. These include the identification of an antigenic trigger(s), the implications (if any) of IgG4 antibodies for pathophysiology and the precise immunological mechanisms leading to fibrosis. Recent investigations have also raised the possibility that innate immunity might precede adaptive immunity, thus further complicating the pathological scenario. Here, we aim to review the most recent insights on the immunology of IgG4-RD, focusing on the relative contribution of innate and adaptive immune responses to the full pathological phenotype of this fibrotic condition. Clinical, histological and therapeutic features are also addressed.     

Autoimmune pancreatitis and IgG4-related systemic diseases

Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis that is characterized by lymphoplasmacytic infiltrate, storiform fibrosis, obliterative phlebitis, and increased IgG4⁺ plasma cells. Serum IgG4 levels usually are elevated. Patients with AIP frequently have disease affecting other organs or sites; these tissues show similar histologic changes, including increased IgG4⁺ plasma cell infiltrate and response to corticosteroid therapy. A new clinicopathologic concept of IgG4-related systemic disease (ISD) has been proposed. These diseases often are not limited to the pancreas, and the pancreas may not be involved at all. In this article, we review the literature and our own experience to detail the clinicopathologic features of AIP and extrapancreatic lesions in ISD.     

IgG4-related renal disease: clinical and pathological characteristics

IgG4-related disease is a recently established systemic condition. Tubulointerstitial nephritis is the most common renal manifestation. Glomerular lesions, particularly membranous glomerulonephritis, can develop simultaneously. Some patients present with serological renal dysfunction associated with elevated IgG or IgE levels and hypocomplementemia, while others are incidentally found to have abnormalities in kidneys on imaging. A majority of patients with IgG4-related kidney disease have similar lesions at other anatomical sites, which help us to suspect this condition. Serum IgG4 elevation (>135 mg/dL) is the most, although not entirely, specific marker for the diagnosis. Imaging findings varies from small nodules to bilateral diffuse abnormalities. In addition to the renal parenchyma, the renal pelvis and perirenal adipose tissue can be affected. Histological features include dense lymphoplasmacytic infiltration, storiform or “bird’s eye” fibrosis (highlighted by PAM stain), and IgG4-positive plasma cell infiltration (>10 cells/high-power field and IgG4/IgG-positive cell ratio >40%). Immune complex deposition is detectable in the tubular basement membrane by immunofluorescence and/or electron microscopy. Patients usually respond well to corticosteroids, but highly active diseases may require other immunosuppressive therapies. Further investigations will be required to fully understand pathophysiology underlying this emerging condition.     

Abdominal aortic aneurysm as an IgG4-related disease

The objectives of this study were to evaluate patients with aortic abdominal aneurysm (AAA) with regard to immunoglobulin (Ig)G4-related disease (IgG4-RD). IgG4-RD represents a recently defined condition comprised of a collection of disorders characterized by IgG4 hypergammaglobulinemia, the presence of IgG4-positive plasma cells in organs affected with fibrotic or sclerotizing changes and typical histopathological features. It was identified as a possible cause of vasculitis in large vessels. Studies have been published on a possible association between inflammatory aortic or cardiovascular disease and IgG4-RD. We examined 114 patients with AAA requiring surgery in order to identify findings which are characteristic of IgG4-RD. Aneurysm samples from seven patients showed histopathological features consistent with IgG4-RD and the presence of IgG4⁺ plasma cells. Only two of these seven patients showed elevated IgG4 serum levels higher 1·35 g/l. In five of the patients, the concentration of serum IgG4 was lower than 1·20 g/l, with the number of IgG4⁺ plasma cells being higher than 50/high-power field. These findings were consistent with AAA being a heterogeneous group of inflammatory diseases with different pathogenesis.