Churg-Strauss综合征发病机制研究进展

Churg-Strauss综合征(CSS)是一种常伴有哮喘的系统性血管炎.迄今为止,CSS的病因和发病机制仍不甚明了.外周血和组织中嗜酸性粒细胞(EOS)增多及EOS代谢产物水平升高,表明EOS参与CSS发病.此外,T细胞分泌细胞因子和致炎因子大量释放,提示T细胞激活."抗中性粒细胞胞浆抗体(ANCA)-细胞因子-序列理论"认为CSS的发病与ANCA和中性粒细胞也有关.     

Syndrome de Churg-Strauss

Churg–Strauss syndrome (CSS) is a granulomatous, necrotizing vasculitis affecting small-sized blood vessels. The disease is characterized by asthma, eosinophilia and extra-respiratory manifestations. ANCA are found in one-third to half of the patients with CSS. The prognosis depends on whether there is cardiac and/or gastrointestinal involvement. Steroids are indicated in every case; in severely affected cases, an immunosuppressive agent, e.g. cyclophosphamide, should be prescribed as well. Nevertheless, the prognosis of CSS remains very good; the remission rate approaches 90%.     

Churg-Strauss Syndrome with pleural involvement

A 51-year-old Japanese man with Churg-Strauss Syndrome (CSS) diagnosed by pleural biopsy is described. He was hospitalized because of high fever and bilateral knee, elbow and shoulder joint pain. Chest roentgenogram and chest computed tomography (CT) scan revealed bilateral massive pleural effusion. Pleural biopsy revealed eosinophilic infiltration and necrotizing granulomas. He was treated with oral prednisolone and his symptoms improved. This is the first report of CSS diagnosed by pleural biopsy.     

变应性肉芽肿性血管炎研究新进展

变应性肉芽肿性血管炎是一种以支气管哮喘(简称哮喘)、血管外坏死性肉芽肿、外周血嗜酸粒细胞增多和组织嗜酸粒细胞浸润为特征的系统性小血管炎,1951年由Churg和Strauss首先报道,故称之为Churg-Strauss综合征,即CSS.     

Churg-Strauss Syndrome Associated with Montelukast Therapy

Churg-Strauss syndrome is a rare form of eosinophilic vasculitis associated with asthma. Several cases of eosinophilic conditions including Churg-Strauss syndrome have recently been reported in asthmatic patients being treated with antileukotriene receptor antagonists. However, whether these drugs have a direct pathogenic role remains controversial. We describe two patients who developed Churg-Strauss syndrome after starting treatment with montelukast.     

Cryopyrin-Associated Periodic Syndrome: An Update on Diagnosis and Treatment Response

Cryopyrin-associated periodic syndrome (CAPS) is a rare hereditary inflammatory disorder encompassing a continuum of three phenotypes: familial cold autoinflammatory syndrome, Muckle-Wells syndrome, and neonatal-onset multisystem inflammatory disease. Distinguishing features include cutaneous, neurological, ophthalmologic, and rheumatologic manifestations. CAPS results from a gain-of-function mutation of the NLRP3 gene coding for cryopyrin, which forms intracellular protein complexes known as inflammasomes. Defects of the inflammasomes lead to overproduction of interleukin-1, resulting in inflammatory symptoms seen in CAPS. Diagnosis is often delayed and requires a thorough review of clinical symptoms. Remarkable advances in our understanding of the genetics and the molecular pathway that is responsible for the clinical phenotype of CAPS has led to the development of effective treatments. It also has become clear that the NLRP3 inflammasome plays a critical role in innate immune defense and therefore has wider implications for other inflammatory disease states.     

Postural Orthostatic Tachycardia Syndrome (POTS): An Update for Clinical Practice

Postural Orthostatic Tachycardia Syndrome (POTS) is a variant of autonomic cardiovascular disorder characterized by an excessive increase in heart rate upon standing associated with light-headedness, headaches, chest pain, shortness of breath, and brain fog. The etiology of POTS is largely unknown and often debilitating. The 3 major hypotheses about the pathophysiology of POTS are autoimmunity, abnormally increased sympathetic activity, and sympathetic denervation leading to central hypovolemia and reflex tachycardia. Given its heterogeneous nature, it is crucial to understand each component of POTS with more emphasis on incorporating a multidisciplinary approach to control the symptoms. Future works should focus on better understanding the POTS pathophysiology and designing randomized controlled trials for implementing effective therapy. In this review, we outline the extent of the problem, studies and resources needed to address the issue, and the diagnostic and therapeutic updates on POTS.     

An Update on Stem Cell Therapies for Acute Coronary Syndrome

Well into the second decade since its conception, cell transplantation continues to undergo intensive evaluation for the treatment of myocardial infarction. At a mechanistic level, its objectives remain to replace lost cardiac cell mass with new functioning cardiomyocytes and vascular cells, thereby minimizing infarct size and scar formation, and improving clinical outcomes by preventing adverse left ventricular remodeling and recurrent ischemic events. Many different cell types, including pluripotent stem cells and various adult-derived progenitor cells, have been shown to have therapeutic potential in preclinical studies, while early phase human trial experience has provided divergent outcomes and fundamental lessons, emphasizing that there remain key issues to address and challenges to overcome before cell therapy can be applied to wider clinical practice. The purpose of this review is to provide a balanced update on recent seminal developments in this exciting field and look to the next important steps to ensure its forward progression.     

A Case with Multiple Colonic Ulcers Simulating Churg-Strauss Syndrome

Abstract: Churg-Strauss syndrome (CSS), a relatively rare disorder which is associated with serious complications, has a highly variable course and several possible manifestations. We present the case of a 35-year-old woman with a history of bronchial asthma, admitted for evaluation of lower abdominal pain and melena, whose clinical course had certain features in common with CSS. On admission, the white blood cell count was 45,300/mm³ with 65% eosinophils, and the serum immunoglobulin E (5,300 u/ml) level was remarkably elevated. At colonoscopy, there were shallow ulcers, irregular in shape, throughout the large intestine. Abdominal pain and melena were relieved by oral administration of prednisolone. Most previously reported cases have not been recognized as having colonic involvement until surgery or autopsy. In only a few reports of CSS and related disorders were colonoscopic examination findings described.     

Pre-eclampsia: An Update

Pre-eclampsia remains the second leading direct cause of maternal death, >99 % of which occurs in less developed countries. Over 90 percent of the observed reduction in pre-eclampsia-related maternal deaths in the UK (1952–2008) occurred with antenatal surveillance and timed delivery. In this review, we discuss the pathogenesis, diagnostic criteria, disease prediction models, prevention and management of pre-eclampsia. The Pre-eclampsia Integrated Estimate of RiSk (PIERS) models and markers of angiogenic imbalance identify women at incremental risk for severe pre-eclampsia complications. For women at high risk of developing pre-eclampsia, low doses of aspirin (especially if started <17 weeks) and calcium are evidence-based preventative strategies; heparin is less so. Severe hypertension must be treated and the Control of Hypertension In Pregnancy (CHIPS) Trial (reporting: 2014) will guide non-severe hypertension management. Magnesium sulfate prevents and treats eclampsia; there is insufficient evidence to support alternative regimens. Pre-eclampsia predicts later cardiovascular disease; however, at this time we do not know what to do about it.     

Prosthesis-Patient Mismatch: An Update

Prosthesis-patient mismatch (PPM) is present when the effective orifice area of the inserted prosthetic valve is too small in relation to body size. Its main hemodynamic consequence is to generate higher than expected gradients through normally functioning prosthetic valves. The purpose of this review is to present an update on the present state of knowledge with regard to diagnosis, prognosis, and prevention of PPM. PPM is a frequent occurrence (20% to 70% of aortic valve replacements) that has been shown to be associated with worse hemodynamics, less regression of left ventricular hypertrophy, more cardiac events, and lower survival. Moreover, as opposed to most other risk factors, PPM can largely be prevented by using a prospective strategy at the time of operation.