IgG4相关性肺疾病二例并文献复习

目的 总结IgG4相关性肺疾病的临床特点及诊治经验,提高临床对该病的认识,减少误诊误治.方法 分析第二军医大学附属长海医院呼吸内科收治的两例IgG4相关性肺疾病的一般情况、临床表现、实验室检查、组织病理学、治疗及预后,并进行相关文献复习.结果 两例患者均为男性,血清IgG4水平均显著升高(2.25 g/L;10 g/L).1例肺部影像学呈实性结节型改变,并有回盲部受累,糖皮质激素治疗有效.另1例肺部影像学呈支气管血管束型改变,对糖皮质激素治疗依赖.文献复习69例IgG4相关性肺疾病,因呼吸道症状就诊者39例(56.5％),伴有肺外受累者41例(59.4％).48例检测了血清IgG4水平,均显著升高(307 ～ 52 500 mg/L).胸部影像学表现实性结节型35例(50.7％).31例(44.9％)患者接受了糖皮质激素治疗,预后良好.结论 IgG4相关性肺疾病是临床少见的免疫性疾病,临床表现缺乏特异性,临床诊断应综合分析其血清免疫学检查、影像学表现及组织病理学结果,糖皮质激素治疗效果良好.     

IgG4相关间质性肺疾病

IgG4相关间质性肺疾病的提出是基于对系统性IgG4相关硬化性疾病的研究发现.IgG4相关间质性肺疾病的诊断需要临床医师结合临床-影像学-病理学特征疑及此病.IgG4相关间质性肺疾病对糖皮质激素治疗反应良好,监测随访对于发现复发是必要的.     

IgG4相关性疾病

IgG4相关性疾病是一种与IgG4淋巴细胞密切相关的慢性、系统性疾病,该类疾病以血清IgG4水平升高以及IgG4阳性细胞浸润多种器官和组织为特征,常见受累器官包括泪腺、胰腺和腹膜后间隙等,累及的器官或组织由于慢性炎症及纤维化进程可导致弥漫性肿大。该类疾病对皮质激素治疗反应良好。     

IgG4相关疾病的实验室特征分析

目的探讨IgG4相关性疾病的共性和有意义的辅助诊断指标。方法回顾性分析福建医科大学附属第一医院2009年5月-2012年12月住院的10例Ig G4相关性疾病患者的临床资料。结果 10例患者中男7例,女3例,发病年龄41~75岁,平均年龄(55.8±13.6)岁,临床表现以黄疸(7/10)和体质量下降(4/10)为主,常见受累器官为胰腺(6/10)和胆管(5/10),均表现为多器官受累,患者血清IgG4均明显升高,影像学检查发现受累脏器呈弥漫性肿大或压迫狭窄,组织病理学检查表现为大量IgG4阳性淋巴细胞浸润并伴有明显纤维化改变,激素治疗后临床症状、影像学和实验室指标均有不同程度改善。结论 IgG4相关性疾病临床症状无特异性,血清IgG4可作为疑似病例的筛查方法。     

IgG4相关性心包炎的临床研究

目的通过报道少见的IgG4相关性心包炎并进行文献复习,分析该病的临床表现和预后。方法报道一例IgG4相关性心包炎,并以"IgG4"和"pericarditis"检索Pub Med进行文献复习。结果本例患者经过临床表现、血清学检查和影像学检查,确诊为IgG4相关性心包炎。IgG4相关性心包炎的临床表现多样,多数经手术病理确诊,对激素治疗反应良好。结论本病临床少见,遇到有心包炎临床特征的患者,可行IgG4血清学检测及其他相关腺体疾病筛查,如合并自身免疫相关性疾病应考虑本病可能,早发现,早诊断,早期糖皮质激素治疗可避免手术治疗。     

IgG4相关性消化疾病的研究进展

IgCA相关性疾病是一种新认识的纤维炎性疾病,其特征是肿块样病灶、以IgG4阳性浆细胞为主的致密的淋巴浆细胞的浸润、受累组织呈轮辐状纤维化,通常伴有血清IgG4升高。自身免疫性胰腺炎（autoimmune pancreatitis,AIP）被认为是一种IgG4相关性系统性疾病,最早是由Yoshida等在1995年提出,     

A case developing minimal change disease during the course of IgG4-related disease

We describe a 66-year-old male with immunoglobulin G4-related disease (IgG4-RD) presenting with minimal change disease (MCD). Three years prior to this admission, the patient had been diagnosed with IgG4-RD. The development of sudden massive proteinuria (4+; 16.7 g/gCr) with a weight gain of 8 kg within a two-week period was noted, and nephrotic syndrome was suspected. The patient's serum IgG4 level did not increase and hypocomplementemia was not found. A renal biopsy showed no cellular infiltration in the renal interstitium, and no spiking or bubbling was found on periodic acid methenamine silver staining. On electron microscopy, foot process effacement was seen, but no subepithelial electron-dense deposits were found. The patient was diagnosed with MCD. Ten days after starting prednisolone (60 mg/day), proteinuria was negative. Since IgG4-RD and MCD share a T-helper 2-dominant immunoreaction, the development of MCD in IgG4-RD patients may reflect more than a mere coincidence.     

IgG4-related lung disease with atypical CT imaging: a case report

IgG4-related lung disease is a rare disease, diagnosed when typical pathologic features are seen in the context of increased serum levels of IgG4 and the elevated tissue’s IgG4-positive plasma cells. Here we reported the case of a 24-year-old woman with IgG4-related lung disease. This patient presented with fever, cough and shortness of breath. Thoracic computed tomography (CT) images demonstrated multiple nodules or masses with high density in both lungs, and thickened interlobular septa. The ‘halo sign’ was observed around the high-density lesions of the upper lobes. This range of CT images’ characteristics is atypical, which differs from previous reports of this condition.     

IgG4-related respiratory disease

Abstract

IgG4-related diseases (IgG4-RDs), such as autoimmune pancreatitis and IgG4-related Mikulicz disease, are often accompanied by intrathoracic lesions, which are called IgG4-related respiratory disease (IgG4-RRD). IgG4-RRD has few subjective symptoms, and is usually detected during workup of patients with extra-thoracic lesions of IgG4-RD. IgG4-RRD is characterized by various conditions, including masses, nodules, thickening, and infiltration at numerous sites in the thorax through lymphatic routes. Although elevated serum IgG4 concentrations and pathologic evidence of lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells are characteristic findings of IgG4-RD, other intrathoracic diseases, such as multicentric Castleman disease and malignancy, may present with similar findings. Developing diagnostic criteria for IgG4-RRD, including clinicoradiological and pathological characteristics, is necessary for its appropriate diagnosis.     

Multicentric Castleman disease mimicking IgG4-related disease: A case report

A 50-year-old woman was referred to our hospital for shoulder joint stiffness. She had a history of polyclonal hypergammaglobulinemia and an elevated C-reactive protein level. Her laboratory data revealed an elevated serum immunoglobulin G4 (IgG4) level, hypergammaglobulinemia, and rheumatoid factor positivity in the absence of anticyclic citrullinated peptide antibody. [18^F]-Fluorodeoxyglucose positron emission tomography showed significant [18^F]-fluorodeoxyglucose uptake in multiple lymph nodes (axillary, hilar, para-aortic, and inguinal). Biopsy of the inguinal lymph node showed expansion of the interfollicular areas by heavily infiltrating plasma cells, consistent with multicentric Castleman disease (MCD). Immunohistochemical analysis revealed a 37.3% IgG4-positive:IgG-positive plasma cell ratio, indicating overlapping IgG4-related disease. However, serological cytokine analysis revealed elevated levels of interleukin-6 (9.3 pg/ml) and vascular endothelial growth factor (VEGF) (1210 pg/ml), which are compatible with MCD. Corticosteroid treatment resolved the serological and imaging abnormalities. IgG4-related disease can mimic MCD, and it is crucial to distinguish between these two diseases. Serum interleukin-6 and VEGF levels may help to discriminate MCD from IgG4-related disease.     

Immunoglobulin G4-related lung disease: A disease with many different faces

Immunoglobulin (Ig) G4-related lung disease is a fibroinflammatory entity that presents in protean ways. Diagnostically, IgG4-related lung disease requires a high clinical index of suspicion complemented by elevated serum IgG4 levels and/or biopsy that shows the characteristic pathological features. The disease is almost always responsive to systemic corticosteroids. However, relapse is common following their discontinuation. The authors present three cases to highlight the diverse clinical features, and to illustrate the diagnostic and therapeutic approaches to this disease.     

Thoracic manifestations of IgG4-related disease

Immunoglobulin G4-related disease (IgG4-RD) is a recently described rare systemic fibroinflammatory disease with an estimated incidence of less than 1 in 100,000 persons per year. The disease can affect virtually any organ and is characterized by unifying histopathological findings. Recently, four subgroups of patients have been characterized: hepatobiliary, head and neck, Mikulicz syndrome and retroperitoneal fibrosis, who illustrate the mainly abdominal and ENT tropism of the disease. Yet, thoracic involvement is not uncommon. It can be detected in up to 30% of patients with systemic IgG4-RD and is the exclusive manifestation of the disease in about 10% of cases. Clinical symptoms are nonspecific and may include dyspnoea, cough or chest pain. Chest CT findings are heterogeneous and primarily include peribronchovascular thickening, nodules, ground-glass opacities and lymphadenopathy. There is no specific diagnostic test for IgG4-RD thoracic involvement, which may mimic malignancy or vasculitis. Therefore, a cautious approach is needed to make an accurate diagnosis: a search for extra-thoracic manifestations, elevated serum IgG4 levels, circulating levels of plasmablasts and pathologic evidence of disease is warranted. Although very suggestive, neither the presence of a polyclonal IgG4 lymphoplasmacytic infiltrate, storiform fibrosis or obliterative phlebitis are sufficient to confirm the histological diagnosis. Steroids are recommended as first-line therapy. Rituximab or disease-modifying antirheumatic drugs may be used in relapsed or rare cases of steroid-refractory disease. In this review, we summarize current knowledge regarding the pathophysiology, epidemiology, diagnostic modalities (clinical–biological–imaging–histopathology) and treatment of IgG4-RD thoracic involvement.