IgG4相关性疾病4例报告并文献回顾

目的 探讨IgG4相关性疾病的临床表现、病理学特征及影像学表现。 方法 分析4例IgG4相关性疾病的临床特征、组织病理学及影像学改变,并查阅相关文献进行总结。 结果 本组4例的平均年龄为55岁,男女比例为3∶1。分别发生于颌下腺2例、眼眶1例、颜面部1例。临床表现无显著特异性,均呈无痛渐进性肿胀。病理学改变为淋巴组织及纤维组织增生,其内有较多浆细胞浸润(IgG4阳性浆细胞>50个/HPF,IgG4阳性浆细胞>IgG阳性浆细胞的40%)。影像学上表现为病变区炎性改变。 结论 IgG4相关性疾病是目前临床较为少见的疾病,发病机制尚不明确。需结合临床表现、血清学检测、病理学特征及影像学表现做出最终诊断,进而行及时准确的治疗。     

Blau综合征成年患者抗青光眼手术后并发白内障手术治疗1例并文献复习

目的 观察1例既往确诊Blau综合征11年的成年患者先后出现多种眼部病变后手术治疗的效果,为规范化诊疗、围手术期管理提供思路及参考。 方法 通过报告1例Blau综合征成年患者病程中先后出现双眼葡萄膜炎、双眼继发性闭角型青光眼、双眼并发性白内障,并分别于2016年左眼行青光眼阀管植入+异体巩膜移植+前房成形+瞳孔再造术、2020年行左眼超声乳化白内障吸除联合人工晶状体植入及虹膜周边切除术的病例,分析和总结Blau综合征患者并发性白内障的临床诊疗思维、围手术期管理等。 结果 患者术后3个月、6个月复查视力明显提高,眼压正常,前房中深,虹膜周切口通畅,人工晶状体位正。 结论 Blau综合征患者的眼部病变需要早期、持续的观察和监测,及时给予规范合理的诊疗后患者可保留足够的视力以保障生活质量。     

慢性鼻-鼻窦炎伴或不伴鼻息肉患者鼻内镜手术的疗效及影响因素分析

目的 探讨慢性鼻-鼻窦炎(CRS)伴或不伴鼻息肉患者鼻内镜手术的疗效及其影响因素。 方法 纳入126例伴鼻息肉和154例不伴鼻息肉的CRS患者,行鼻内镜手术治疗后随访6个月,比较两组患者的疗效、并发症和生活质量,并分析疗效的影响因素。 结果 两组疗效和并发症发生率差异无统计学意义(P>0.05);治疗后CRS伴或不伴鼻息肉患者的生活质量SF-36问卷躯体健康、躯体角色功能、心理健康、情绪角色功能、精力和总体健康得分均较前显著升高(P<0.05),CRS不伴鼻息肉患者的情绪角色功能显著高于CRS伴鼻息肉患者(t=-4.107, P<0.001);治疗后规律冲洗鼻腔(P<0.001, OR=0.456, 95%CI: 0.727~0.913)和规律使用激素(P=0.029, OR=0.645, 95%CI: 0.325~0.752)对疗效有显著影响。 结论 鼻内镜手术对CRS伴或不伴鼻息肉患者的疗效和安全性无差别,但不伴鼻息肉的患者术后情绪角色功能恢复更佳,CRS的疗效还受到术后是否规律行鼻腔冲洗和激素的影响,因此临床应重视术后处理。     

肺炎克雷伯杆菌性肝脓肿眼内炎31例

目的 探讨肺炎克雷伯杆菌性肝脓肿眼内炎的临床特征。 方法 收集来自眼科、肝胆外科、内分泌科、血液内科及呼吸内科收治的肺炎克雷伯杆菌性肝脓肿眼内炎的患者共31例(37眼)临床资料,分析患者一般资料、眼部查体、辅助检查、治疗及预后情况。 结果 患者主要为中老年男性,多数合并糖尿病。10眼无眼部症状,在行眼部检查时发现眼内炎表现。22眼行玻璃体腔注药术,12眼行玻璃体切除联合硅油填充术,10眼经治疗后视力提升,6眼最终行眼内容剜除术。 结论 肺炎克雷伯杆菌性肝脓肿眼内炎早期症状不典型,糖尿病及全身感染较重是其危险因素。部分患者早期无眼部症状,在行眼科检查时发现眼内炎表现,及时给予有效治疗从而得以保留较好视力。对无眼部症状的肝脓肿患者行眼科检查对于发现无症状眼内炎患者并给予及时有效治疗有重要意义,有助于保存视力,改善预后。     

继发性甲旁亢甲状旁腺切除不同移植方案疗效研究

目的 回顾性总结继发性甲状旁腺功能亢进症患者行甲状旁腺切除移植术不同移植方案的疗效。 方法 2013年3月至2018年3月共86例甲状旁腺全切移植术患者,按先后不同3种移植方案分为3组,收集患者术前、术后以及随访PTH结果、住院天数、术后补钙天数、术后并发症和复发情况等判断疗效。 结果 3组短期(术后1 d~1个月)治疗效果有差别,而长期治疗效果差异不大,其中A组短期疗效最差,B组、C组短期治疗效果较为接近,但总体而言C组好于B组。 结论 移植甲状旁腺颗粒大小、移植床血供对移植的甲状旁腺存活和生长有着密切关系。从手术角度处理难治性继发性甲状旁腺功能亢进,将甲状旁腺组织小颗粒的方式移植于胸锁乳突肌肉囊,是一种简便、易行、高效的方式。     

视网膜光凝联合玻璃体切除术治疗增生性糖尿病视网膜病变的有效性及安全性

目的 探讨视网膜光凝(RP)联合玻璃体切除术治疗增生性糖尿病视网膜病变(PDR)的有效性及安全性.方法 回顾性分析2010年3月至2014年3月行RP联合玻璃体切除术的79例(79眼)PDR患者的临床资料,观察患者术后视力改善及手术并发症情况.结果 术后随访12个月,视力提高61眼(77.22%),视力不变15眼(18.99%),下降3眼(3.80%).玻璃体切除术后1个月内有2眼发生眼压升高超过30 mmHg,1眼出现新生血管性青光眼(NVG)、1眼出现视网膜脱离; 3个月内有2例出现玻璃体再出血; 12个月内有2眼发生硅油乳化继发青光眼.结论 玻璃体切除术后造影能够有效治疗RP,既能避免过度光凝,又能防止光凝不足,在提高患者视力的同时,可减少并发症,提高安全性,值得临床推广.     

累及喉部的进行性结节性组织细胞增生症1例并文献复习

目的 探讨1例罕见的累及喉部的进行性结节性组织细胞增生症的诊疗过程、临床及病理特点。 方法 提取患者的病例资料、病理切片,对切片进行免疫组化染色观察,总结本病历的特点并查询PNH相关文献。 结果 本病历中患者的病理提示梭形细胞弥漫浸润,免疫组化显示CD68和CD163阳性。患者出现声门上狭窄、气管壁增厚、气管内多发结节,曾因呼吸困难行气管切开,累及皮肤形成多发结节,面部结节形成“狮面”外观,确诊后予以安罗替尼药物治疗可减缓病情发展。 结论 该病通过病理检查确诊,可累及喉部引起呼吸困难,面部结节除非严重影响功能,不主张手术切除皮损。     

低温等离子射频技术在婴幼儿甲状舌管囊肿手术中的应用

目的 探讨低温等离子射频技术在婴幼儿喉内型甲状舌管囊肿手术切除中的应用.方法 实施低温等离子手术治疗由于喉内型甲状舌管囊肿引起上呼吸道梗阻的婴幼儿患者9例,回顾性分析临床资料,结合相关文献进行总结讨论.结果 9例患者均手术成功,无术后出血、感染等并发症,患者术后随访1年呼吸道梗阻消失,未复发.结论 低温等离子技术应用于婴幼儿喉内型甲舌囊肿切除术时短,创伤小,并发症发生率低.     

变应性鼻炎患者外周血EOS-CSF、IL-5水平及EOS数目对局部皮质类固醇激素治疗效果的评价

目的 检测持续性中重度变应性鼻炎(AR)患者局部应用皮质类固醇激素治疗前后外周血中嗜酸粒细胞集落形成因子(EOS-CSF)、白介素5(IL-5)的水平,判断AR症状缓解以及临床药物治疗效果评价的方便快速的实验室指标。方法 研究分AR组、AR治疗4周组、AR治疗12周组及正常对照组。采用ELAISA方法检测外周血EOS-CSF、IL-5水平,常规计数外周血嗜酸粒细胞(EOS)。应用视觉模拟量表(VAS)评价患者的鼻部症状。结果 AR组血中EOS-CSF和IL-5水平及外周血中EOS数目明显高于正常对照组。AR治疗4、12周组与AR相比,IL-5和EOS-CSF水平及外周血中EOS数目都明显降低。患者血清IL-5水平与EOS计数呈正相关(r=0.63, P<0.05);血清EOS-CSF水平与EOS计数呈正相关(r=0.68, P<0.05)。结论 经鼻用类固醇激素治疗后,检测外周血EOS-CSF和IL-5及查外周血EOS数目可用以监测变应性鼻炎治疗效果并对调整用药提供指导。     

变应性鼻炎伴哮喘患者经微波治疗后的肺功能及呼吸阻力改变

目的 探讨变应性鼻炎伴哮喘患者经微波治疗后,肺通气功能及呼吸阻力受到的影响和变化。 方法 选取变应性鼻炎伴哮喘患者43例,分别在治疗前后对所有患者进行肺功能检测。微波治疗,功率选择30 W,启动时间4 s,针对下鼻甲黏膜下、中鼻甲前段以及鼻丘进行微波多点凝固。 结果 总有效率100%,显效30例(69.8%)、有效13例(30.2%)。未发生不良反应或并发症。一秒用力呼气量(FEV1)、一秒呼气/用力肺活量(FEV1%)、用力呼气量75%流速(FEF75)的改善差异有统计学意义(P<0.05),用力呼气量25%流速(FEF25)、用力呼气量50%流速(FEF50)的改善差异无统计学意义(P>0.05)。 结论 微波治疗变应性鼻炎伴哮喘患者,肺通气功能有效改善,呼吸阻力降低。     

A novel concept of Mikulicz's disease as IgG4-related disease

Since Morgan's report in 1953, Mikulicz's disease (MD) has been considered part of primary Sjögren's syndrome (SS). However, MD has a unique presentation, including persistent swelling of the lacrimal and salivary glands, and is characterized by good responsiveness to glucocorticoids, leading to recovery of gland function. Recently, it has been revealed that MD patients show elevated serum immunoglobulin G4 (IgG4) levels and prominent infiltration of IgG4-positive plasmacytes. The complications of MD include autoimmune pancreatitis, retroperitoneal fibrosis, tubulointerstitial nephritis, autoimmune hypophysitis, and Riedel's thyroiditis, all of which show IgG4 involvement in their pathogenesis. Thus, MD is a systemic “IgG4-related disease.” In addition, recent analyses have revealed that Küttner's tumor (KT), a chronic sclerosing sialadenitis that presents with asymmetrical firm swelling of the submandibular glands, is also associated with prominent infiltration of IgG4-positive plasmacytes. MD and KT differ from SS and are thought to be singular systemic IgG4-related plasmacytic diseases. Here we discuss the results of recent studies and provide an overview of MD as an IgG4-related disease.     

Otologic Manifestation in IgG4-Related Systemic Disease

IgG4-related systemic disease, including autoimmune pancreatitis, is a multi-organ disorder characterized by elevated serum immunoglobulin G4 (IgG4) concentration and IgG4-positive plasma cell infiltration. We report a case of a 66-year-old woman with IgG4-related hearing disorder, presenting with fluctuating mixed hearing loss with middle ear effusion. The serum IgG4 level was elevated and pathological examination revealed dense infiltration of IgG4-positive lymphocyte in the renal parenchyma, lung tissue and lacrimal gland. With intravenous methylprednisolone at a dose of 60 mg daily, improvement of hearing loss were observed. No recurrence was observed for 6 months with mainternance of prednisolone and methotrexate.     

IgG4-related disease presenting as otogenic skull base osteomyelitis

IgG4-related disease (IgG4-RD) is an emerging clinical disease entity characterized by tumefactive lesions at multiple sites with a dense lymphoplasmacytic infiltrate rich in IgG4+ plasma cells. Although almost any organ can be affected, IgG4-RD is most likely to involve the submandibular, lacrimal, or parotid glands in the head and neck region. However, skull base involvement presenting as otogenic skull base osteomyelitis (SBO) is rare. We encountered a 70-year-old male with IgG4-RD presenting primarily with severe otalgia and otorrhea. He had uncontrolled diabetes mellitus and showed clinical manifestations of otogenic SBO. Tissue immunostaining revealed typical features of increased IgG4-positive plasma cells, and hematological examination showed elevated serum IgG4 concentrations. Treatment with corticosteroids significantly improved well-being and partially resolved the lesion based on computed tomography (CT) scan.     

A solely ear-involved IgG4-related sclerosing disease with two-years following-up

IntroductionImmunoglobulin G4-related sclerosing disease (IgG4-RSD) is a chronic fibro-inflammatory disease involving systemic multi-organ lesions, such as the salivary and lacrimal glands, lymph nodes and pancreas. The diagnosis of this disease is reliant upon clinical manifestations, laboratory tests, histopathologic results and radiological data. Some studies have found that IgG4-RSD has otological manifestations, whereas there were few studies introducing the diagnosis, therapy and long-term follow-up results of solely otological IgG4-RSD.Case summaryHere, we report a case of IgG4-RSD involving the ear alone. A female presenting with otalgia and hearing loss underwent surgery, without hormone therapy. The pathological diagnosis was otological IgG4-RSD and no clinical or radiological signs of recurrence were observed at seven and twenty-four months follow-up.DiscussionThis case indicates that IgG4-RSD can invade the ear only, and that surgical therapy without hormone therapy is efficient for solely IgG4-RSD. Pathological results are crucial for diagnosis.     

Warthin's tumor associated with IgG4-related disease

Immunoglobulin G4-related disease (IgG4-RD) is an inflammatory condition associated with elevated serum IgG4 levels and tissue infiltration by IgG4-expressing plasma cells. Several inflammatory conditions associated with IgG4-RD have been reported. Warthin's tumor is a benign parotid gland tumor consisting of oncocytic epithelial cells and lymphoid stroma containing lymphoid follicles with reactive germinal centers. This is the first report describing a case of Warthin's tumor with possible involvement of IgG4-RD. The patient was a 71-year-old man presenting with swollen right parotid and bilateral submandibular glands. He had a history of a Warthin's tumor of the left parotid gland, autoimmune pancreatitis, and an inflammatory abdominal aortic aneurysm. Laboratory tests revealed a high serum IgG4 level. Histological examination of the resected parotid gland showed a Warthin's tumor and a nodule showing severe lymphocytic infiltration containing many IgG4-positive plasma cells. This case shows the possible involvement of Warthin's tumor with IgG4-RD.     

IgG4-related disease with pseudotumor formation in the larynx

Mikulicz’s disease, an immunoglobulin G4-related disease (IgG4-RD) occurs frequently in the head and neck region but rarely in the larynx. We report a case of IgG4-RD with pseudotumor formation in the larynx. A 50-year-old man presented at our facility for a complete physical examination and diagnostic testing of a left arytenoid tumor. On examination, a large supraglottic mass was noted with signs of dyspnea. Movement disorder of the vocal fold was absent. The patient underwent surgery with general anesthesia to improve respiratory symptoms and a resected specimen was submitted for diagnosis. The pathology findings revealed lymph follicle formation, interstitial follicular fibrosis, angiogenesis, and inflammatory cell infiltration with plasmacytosis. Immunohistologic staining at high magnification revealed one hundred or more IgG4-positive plasma cells and fifty percent IgG4/IgG. In addition, obliterative phlebitis was observed. Medical history was positive for retroperitoneal fibrosis with serum IgG4 levels below the diagnostic criteria of IgG4-RD, but histological characteristics of IgG4-RD were met. The diagnosis of IgG4-RD was established, oral treatment with prednisolone (0.6 mg/kg/day) was initiated, and the tumor reduced in size. IgG4-RD may cause irreversible functional impairment. Early diagnosis and treatment are important in reducing the risk of permanent impairment of vocal fold mobility.     

Mucosa-associated lymphoid tissue lymphoma of the thyroid with abundant IgG4-positive plasma cells

A case of thyroidal mucosa-associated lymphoid tissue (MALT) lymphoma mimicking IgG4-related disease is described. A 54-year-old male presented with acute swelling of the anterior neck. Anaplastic thyroid carcinoma (ATC), malignant lymphoma (ML), or acute deterioration of Hashimoto's thyroiditis were initially suspected, and an emergent tracheostomy was required for progressive airway obstruction; a simultaneous biopsy from the thyroid tissue was performed. Histopathologically, the lesion consisted of sclerotic fibrosis and diffuse and dense infiltration by small lymphoid cells without atypia and plasma cells, many of which were IgG4-positive. Blood examination also revealed high serum IgG4 levels. Riedel's thyroiditis was suspected. However, despite medical treatments, a firm swelling of the thyroid still remained. In an in situ hybridization study, IgG4-negative plasma cells showed immunoglobulin light-chain restriction (κ-monotype), and immunoglobulin heavy (IgH) chain gene monoclonal re-arrangement was detected by polymerase chain reaction. The lesion was finally diagnosed as MALT lymphoma. When IgG4-related disease is suspected, it is important to thoroughly exclude other possibilities.     

Penetration of administered IgG into the maxillary sinus and long-term clinical effects of intravenous immunoglobulin replacement therapy on sinusitis in primary hypogammaglobulinaemia

The influence of long-term intravenous immunoglobulin (i.v. IgG) replacement therapy on the clinical course of chronic sinusitis in patients with primary hypogammaglobulinaemia has not previously been reported. We have analysed the efficacy of i.v. IgG therapy and the penetration of administered i.v. IgG into the maxillary sinus. Seventeen patients with primary hypogammaglobulinaemia received i.v. IgG replacement therapy to maintain pre-infusion serum IgG concentrations above 4 g/l for periods of 12 to 58 (mean 36.7) months. Cases with established chronic sinusitis prior to therapy did not have symptomatic or radiological improvement at this dose, although no sinusitis developed de-novo in the 3 previously unaffected patients. The administered IgG penetrated into maxillary sinus antral lavage fluid in 3 patients from whom secretions were obtained at antroscopy. This indicates that poor clinical responses are not due to lack of penetration of antibodies to the required sites of action. Larger doses of i.v. IgG may be more effective in this situation, but the addition of antibiotics at high dosage may be a more economical therapeutic alternative. These findings highlight the importance of diagnosis of hypogammaglobulinaemia by measurement of serum IgG concentrations in patients who suffer from recurrent sinusitis, as the early institution of i.v. IgG therapy may prevent the development of sinusitis refractory to i.v. IgG therapy.     

高压氧疗治疗大前庭导水管综合征患儿急性听力丧失九例

目的 目的探讨高压氧疗治疗大前庭导水管综合征患儿急性听力丧失的临床效果。 方法 对急性听力丧失的大前庭导水管综合征患者9例11耳,进行为期2周共10次高压氧治疗。 结果 患儿均耐受良好,治疗后复查纯音听阈测试,提示患耳听阈均有明显改善。 结论 9例患儿对高压氧疗耐受良好,治疗效果显著,高压氧疗可以成为大前庭导水管综合征急性听力丧失患者的治疗方法之一。