抗中性粒细胞胞质抗体相关性血管炎发病机制及治疗的研究进展

抗中性粒细胞胞质抗体(ANCA)相关性血管炎(AAV)主要以小血管壁的炎性反应和纤维素样坏死为病理表现,伴随血清ANCA阳性,累及全身多系统的自身免疫性疾病。主要临床表现包括:显微镜下多血管炎(MPA),肉芽肿性多血管炎(GPA),嗜酸性肉芽肿性多血管炎(EGPA)等。最常见累及器官包括呼吸道及肾脏,病情凶险,死亡率高。本文旨在综述AAV发病机制及治疗方法的最新研究进展。     

杀菌通透性增强蛋白-抗中性粒细胞胞质抗体相关性血管炎合并支气管扩张1例并文献复习

抗中性粒细胞胞质抗体(anti-neutrophil cytoplasmic antibody, ANCA)相关性血管炎是一组以血清中能够检测到ANCA为突出特点的系统性小血管炎, 杀菌通透性增强蛋白(bactericidal permeability increasing protein, BPI)是ANCA的靶抗原之一。BPI-ANCA相关性血管炎临床上虽并不多见, 但合并支气管扩张并非偶然。该文报道1例BPI-ANCA相关性血管炎肾损害合并支气管扩张, 并复习相关文献, 探讨BPI-ANCA相关性血管炎的病例特点及支气管扩张与ANCA相关性血管炎的相关性, 以提高临床医师对该类疾病的认识。     

中性粒细胞胞外诱捕网在腹主动脉瘤中的研究进展

腹主动脉瘤(AAA)的发病机制复杂, 包括动脉硬化、血流动力学和遗传等多种因素。中性粒细胞在AAA发生发展过程中扮演重要角色, 但具体机制尚未明确。中性粒细胞胞外诱捕网(NETs)是由中性粒细胞发生程序性死亡后释放至胞外参与诱捕细菌的网格样结构。研究结果显示中性粒细胞通过释放NETs参与炎性反应及AAA病理生理过程。本文对NETs参与AAA发生发展的机制研究进行综述。     

中性粒细胞胞外诱捕网与胰腺疾病的研究进展

中性粒细胞胞外诱捕网（NETs）是近年来研究的热点,是中性粒细胞杀灭病原体的一种新的免疫机制。越来越多的研究表明,NETs参与了心血管疾病、胆结石的形成、炎症、肿瘤等疾病的病理生理过程。研究显示,NETs是急性胆源性胰腺炎（ABP）的发病机制之一、是急性胰腺炎（AP）病情进展的重要因素,也是促进胰腺癌细胞的增殖、转移、以及参与胰腺癌患者静脉血栓的形成的关键因素,笔者对此加以综述。     

中性粒细胞细胞外网络在ANCA相关性小血管炎患者肾组织中的表达及意义

目的:研究中性粒细胞细胞外网络(NETs)在ANCA相关性小血管炎(AASV)患者肾组织中的表达并初步探讨其在AASV中的可能致病机制。方法:采用免疫组织化学技术,分别检测NETs(以瓜氨酸化的组蛋白H3为标志)在8例ANCA相关性小血管炎患者,8例微小病变型肾病综合征患者,3例健康对照的肾小球、肾间质及肾小管的表达,同时检测相应部位B淋巴细胞(CD19做标志)的浸润。结果:(1)AASV患者肾小球中NETs表达较微小病变组患者和健康对照组肾小球中NETs表达明显增多[(0.354±0.347)vs(0±0),P<0.05],在AASV肾脏病理损害严重部位,如中、重度系膜细胞增生的肾小球(0.485±0.721),有炎症细胞浸润的肾间质(2.575±2.99)、肾小管(1.417±2.888)中浸润的NETs明显增多。(2)B淋巴细胞(CD19)的表达:AASV患者肾小球球周B淋巴细胞表达较健康对照组[(3.123±4.411)vs(0±0)]、微小病变组[(3.123±4.411)vs(0±0)]表达明显增多,与微小病变组相比差异有统计学意义(P<0.05)。在AASV患者肾组织中主要分布于硬化的肾小球球周(4.024±7.457)、中重度系膜细胞增生的肾小球球周(2.673±2.948),在肾间质炎细胞浸润的区域常成簇聚集(9.625±8.961),而在肾小球内基本无表达。结论:NETs在AASV患者肾组织广泛表达,肾小球中浸润的NETs可能参与了AASV患者的肾损害。     

抗中性粒细胞胞浆抗体相关性血管炎并发脑梗死1例

血管炎是一种以血管的炎症和损伤为特征的临床病理过程,常有血管病变,伴有受累血管供血组织缺血[1]。抗中性粒细胞胞浆抗体(antineutrophil cytoplasmic antibody,ANCA)相关性血管炎是一组累及全身多系统的自身免疫性疾病。本病病情复杂,临床特征不具有特异性,容易误诊和漏诊。笔者对1例ANCA相关性血管炎患者的诊治经过报道如下,望为提高临床医生对本病的认识略尽绵力。     

肺结核合并 IgA 肾病及 ANCA 相关性血管炎1例

肺结核是一种常见传染病，常用的抗结核药物为异烟肼、利福平、乙胺丁醇、吡嗪酰胺。IgA 肾病是我国最常见的肾小球疾病，发病机制尚未明确；由于其以免疫球蛋白 A（IgA）在系膜区沉积为主要分子病理特征，提示 IgA 肾病是一种免疫相关性疾病。抗中性粒细胞胞质抗体（ ANCA）相关性血管炎是系统性自身免疫性疾病，肾脏是最易受累的器官，因其临床表现多样，临床上容易误诊和漏诊。肺结核、IgA 肾病、ANCA 相关性血管炎三者同时存在的病例相当少见，现报告1例。     

ANCA相关小血管炎的治疗进展

原发性小血管炎是目前尚未明确病因的一类小血管炎，主要侵犯小血管，如小动脉、细动脉、毛细血管等，以血管壁坏死性炎症、纤维素样坏死为病理特征，是一类自身免疫性疾病。在原发性小血管炎中，部分疾病与抗中性粒细胞浆抗体（ANCA）密切相关，因而称之为ANCA相关小血管炎（AASV），是成人最常见的原发性小血管炎，包括显微镜下型多血管炎（MPA）、韦格纳氏肉芽肿（WG）及过敏性肉芽肿性血管炎（CSS）。     

抗中性粒细胞胞浆抗体相关性系统性血管炎发病机制研究进展

抗中性粒细胞胞浆抗体（ANCA）相关性系统性血管炎（AASV）是一类由遗传、环境及免疫等诸多因素共同作用所致的系统性自身免疫性疾病,它包括韦格纳肉芽肿（WG）,显微镜下多血管炎（MPA）和过敏性肉芽肿性血管炎（churg-strauss syndrome）等。有关AASV的发病机制至今尚未完全清楚,     

抗中性粒细胞胞质抗体相关性血管炎的研究现状

抗中性粒细胞胞质抗体(ANCA)是存在于系统性血管炎患者中的一类自身抗体,其抗原是多形核粒细胞和单核细胞的胞质成分.除此之外,微型多动脉炎、局限性肾动脉炎等的发病与ANCA亦密切相关.临床上将上述疾病称为ANCA相关系统性血管炎(AASV).AASV肾脏损害的表现主要包括少尿、血尿和蛋白尿,伴肾小球滤过率下降.目前诊断主要依赖肾脏病理学检查,其特征性病理表现为寡免疫复合物沉积的局灶节段坏死性肾小球肾炎,形成以巨噬细胞和上皮细胞为主要细胞成分的新月体.AASV预后不良的因素包括老年、肾功能严重受损.肾脏病理为判断预后提供更多的依据,肾间质纤维化提示预后不良.     

Azathioprine hypersensitivity: report of two cases and review of the literature

Azathioprine (AZA) is a widely-used drug in the treatment of different diseases such as vasculitis, systemic lupus erythematosus, rheumatoid arthritis, inflammatory bowel diseases and in renal transplantation. Side effects of AZA can be classified as toxic, mainly dose related (myelosuppression and hepatotoxicity) and idiosyncratic, mainly dose independent. While the toxic effects are common and well documented, the hypersensitivity reactions are rare and it is not often easy to distinguish them from systemic sepsis or disease recurrence. We report two cases of AZA hypersensitivity occurring in patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis each mimicking a vasculitis relapse or a septic complication of immunosuppression, as well as a review of the literature.     

鸢尾素在炎性疾病中的抗炎作用

目的总结鸢尾素在炎性疾病中的抗炎作用。方法系统检索并阅读近年来国内外关于鸢尾素抗炎作用研究的相关文献并进行综述。结果鸢尾素在体内分布广泛并发挥着诱导白色脂肪细胞褐变、改善能量代谢、提高葡萄糖利用率等生理学作用。在急性肺损伤、炎症性肠病、脓毒性心肌病、急性胰腺炎、非酒精性脂肪性肝病和恶性肿瘤中通过抑制白细胞介素-6、肿瘤坏死因子-α等炎症因子升高,拮抗细胞凋亡和核因子-κB活化、改善组织损伤等机制发挥重要抗炎作用。结论鸢尾素在炎症性肠病、急性肺损伤等炎性疾病中发挥重要抗炎作用,有望作为炎性疾病诊断和预后的生物标志物并成为炎症疾病治疗的新靶点。     

Management of Severe Acute Pancreatitis

Severe acute pancreatitis (SAP) develops in about 25% of patients with acute pancreatitis. Severity of acute pancreatitis is linked to the presence of systemic organ dysfunctions and/or necrotizing pancreatitis. Risk factors independently determining the outcome of SAP are early multiorgan failure (MOF), infection of necrosis, and extended necrosis (>50%). Morbidity of SAP is biphasic, in the first week it is strongly related to systemic inflammatory response syndrome while, sepsis due to infected pancreatic necrosis leading to MOF syndrome occurs in the later course after the first week. Contrast-enhanced computed tomography provides the highest diagnostic accuracy for necrotizing pancreatitis when performed after the first week of disease. Patients who suffer early organ dysfunctions or are at risk for developing a severe disease require early intensive care treatment. Antibiotic prophylaxis has not been shown as an effective preventive treatment. Early enteral feeding is based on a high level of evidence, resulting in a reduction of local and systemic infection. Patients suffering infected necrosis causing clinical sepsis are candidates for intervention. Hospital mortality of SAP after interventional or surgical debridement has decreased to below 20% in high-volume centers.     

C反应蛋白与白蛋白比值对腹部外科相关疾病预后价值的研究进展

C反应蛋白作为经典的急性反应蛋白,是反映机体炎症反应严重程度的评估指标。白蛋白既可以反映机体的营养状态,也能体现疾病所导致的机体消耗程度。目前,C反应蛋白与白蛋白比值已被证实在腹部外科多种疾病,如急性胰腺炎、炎症性肠病以及各类腹部肿瘤的预后方面都存在一定的预测价值。该指标相较于单独检测C反应蛋白或白蛋白有着更高的灵敏性...     

Pancreatitis and intravenous fat: an association in patients with inflammatory bowel disease

Since recently adding Intralipid (IL) to our standard total parenteral nutrition (TPN) regimen for children with inflammatory bowel (IBD) disease, two children undergoing major intestinal resections have developed acute pancreatitis in the early postoperative period. Herein, we present the clinical summaries of these two patients, discuss the multifactoral etiologies of acute pancreatitis, and review the role of emulsified fat solutions in TPN in children with IBD.     

Potential effects of hyperbaric oxygen therapy in acute pancreatitis

BACKGROUND: To extract from the biomedical published reports, the effects of hyperbaric oxygen (HBO) on inflammatory disease, in particular acute pancreatitis. METHODS: This review will explain these effects and evaluate potential mechanisms of action of HBO in acute pancreatitis. A Medline/PubMed search (January 1966 to July 2004) with manual cross-referencing was conducted, including all relevant articles investigating the molecular and systemic effects of HBO on inflammatory diseases, particularly focusing on the studies of acute pancreatitis. All publication types, languages and subsets were searched. RESULTS: Original and review articles and short communications were extracted. The selected original articles covered the molecular and systemic effects of HBO and the effects in inflammatory disease states. The major findings are that HBO can act as an anti-inflammatory agent and as an antimicrobial agent. Many of the effects of HBO would be beneficial in the treatment of acute severe pancreatitis. Work carried out to date in animal models of acute pancreatitis shows promising improvements in severity but studies are limited to date. CONCLUSION: Acute pancreatitis impairs the pancreatic and systemic microcirculation and causes acute inflammation. These processes are potentially improved by HBO therapy.     

Possible implication of disordered neutrophil extracellular traps in the pathogenesis of MPO-ANCA-associated vasculitis

Neutrophil extracellular traps (NETs) are characterized by the presence of extracellular DNA fibers studded with antimicrobial proteins, including myeloperoxidase (MPO). Although NETs play an important role in the innate immune system, the scattered extracellular enzymes, such as MPO, pose risks to the host. Therefore, NETs are strictly regulated by DNase I in the serum, which prevents them from persisting. Recent studies have demonstrated that dysregulation of NETs could be involved in the pathogenesis of autoimmune diseases, including systemic lupus erythematosus. In this review, we interpret the association of disordered NETs with autoimmune diseases, especially propylthiouracil-induced MPO-ANCA-associated vasculitis.     

Lipid peroxidation in acute surgical diseases of abdominal cavity organs]

The study of lipid peroxidation (LPO) characteristics in patients with acute mesenterial circulatory disturbances (64), acute pancreatitis (44) and acute bowel obstruction (46) allowed the conclusion on informative value of determination of malonic dialdehyde and diene conjugates in the blood for staging these diseases and the degree of severity of endotoxicosis. On the basis of the state of LPO it became possible to evaluate the extent of bowel failure. According to LPO in postoperative period the process of development of vascular rethrombosis in the bowels could be assessed. The varieties of lipid peroxidation products in peripheral venous blood can be used for differential diagnosis of such diseases, similar in their clinical symptoms, as acute pancreatitis and acute bowel obstruction.     

Intestinal ischemia caused by mesenteric veno-occlusive inflammatory disease: description of a clinical case

The Authors report a case of small bowel venous ischaemia due to a peculiar form of vasculitis called Mesenteric Inflammatory Veno-Occlusive Disease. Such disease represents a new anatomopathological entity, with exclusive thrombotic and inflammatory infiltration of the mesenteric veins, with no arterial flogistic involvement. Clinical manifestation of the diseases is that of the venous mesenteric thrombosis in patients in whom the more common forms of systemic vasculitis are not recognizable.