系统性红斑狼疮患者骨质疏松的相关因素

骨质疏松症对于红斑狼疮患者来说是重要的也是可以预防的并发症之一。过去的十几年里,随着红斑狼疮患者生存率的升高,临床医生把更多的精力放在了如何防治红斑狼疮的并发症上,比如骨质疏松。过去的很多代表性研究给我们提供了许多红斑狼疮的流行病学依据,提示患者的骨丢失是一个多因素共同作用的过程,其中包括传统的以及与红斑狼疮疾病本身相关因素。笔者讨论了红斑狼疮患者并发骨质疏松症的一些危险因素以及防治手段。     

系统性红斑狼疮相关骨质疏松的研究进展

近年来,系统性红斑狼疮(systemic lupus erythematosus,SLE)患者的生存率不断提高,生活质量逐渐改善,但骨质疏松(osteoporosis,OP)作为其常见并发症之一,发生率也在逐年增加,给患者的生活、家庭及社会带来了较为沉重的负担和压力。引起SLE骨质疏松的原因很复杂,除传统研究证明的疾病自身活动因素外,药物使用、代谢因素、肾损害、激素水平、生活方式等也参与其发生。在防治过程中,原发病的规范治疗,尤其是包括糖皮质激素在内的药物的合理使用十分关键;联合补充钙剂和维生素D有利于骨矿化、提高骨骼中钙的含量;合理使用双膦酸盐类药物、组织蛋白酶K抑制剂、甲状旁腺激素、狄诺塞麦等药物,可以一定程度上提高骨骼强度,减少骨折的发生;中医药作用的多靶点性对于骨质疏松的治疗也有其独特优势。本文结合临床及新近研究进展,主要就SLE相关骨质疏松的危险因素以及防治等两个方面来予以综述。     

系统性红斑狼疮相关骨质疏松危险因素的研究进展

系统性红斑狼疮(SLE)是累及多个系统的自身免疫性疾病,患者大多是年轻女性,病程较难预测,常常是病情缓解和病情活动交替出现。临床上,SLE合并骨质疏松并不少见,其病理机制复杂,尚无确切定论。骨质疏松也是SLE疾病的并发症之一,但并非单纯由糖皮质激素治疗等原因导致。近年来研究发现,SLE患者骨密度减低与疾病活动和病情进展有关,疾病相关炎症反应、代谢、内分泌、自身抗体、遗传因素及药物影响均参与骨质疏松的发生。因此,本文对SLE相关骨质疏松的研究进展做一综述。     

系统性红斑狼疮相关骨质疏松及其影响因素

近年来,系统性红斑狼疮患者低骨密度和脆性骨折的发病率逐年增加。目前广泛认为系统性红斑狼疮可以增加骨折的风险,并且大量临床研究发现系统性红斑狼疮患者易发生骨质疏松。糖皮质激素的使用是这类患者发生骨质疏松最普遍的原因,但目前证据表明除此之外还有其他相关影响因素。最近的文献已经证明了系统性红斑狼疮患者体内维生素D的水平与骨密度之间有相关性。许多研究也报道了系统性红斑狼疮患者维生素D水平低下或不足的高发生率。维生素D水平低下或不足能导致低骨密度,增加脆性骨折的发生。除此之外,炎症也能改变正常骨代谢,导致骨质和结构的改变。本文也讨论了系统性红斑狼疮相关骨质疏松的预防和治疗策略。     

男性系统性红斑狼疮32例临床分析

本文回顾性分析了３２例男性系统性红斑狼疮（ＳＬＥ）患者，发现男性ＳＬＥ发病年龄较轻，平均２９．４岁，临床特点是脸部蝶形红斑有雷诺现象发现女性低，易误诊：但肾病、脱发、口腔溃疡及胸膜炎发生率较女性更为多见，实验室检查常见因和血小板减少，而血清Ｃ３、ＣＨ５０下降及ＩｇＧ升高发生率明显低于女性。     

系统性红斑狼疮患者骨密度检测分析及对策

为探讨系统笥红斑狼疮（ＳＬＥ）女患者服用糖皮质激素（ＧＣ）后的骨量变化,早期发现骨质疏松（ＯＰ）,减少骨折发生,提高患者生活质量。采用双能量Ｘ线骨密度（ＢＭＤ）测量仪对５０例ＳＬＥ女患者股骨近端和腰椎进行ＢＭＤ测定,并设对照组进行比较和分析。结果?５０例ＳＬＥ患者股骨近端、腰椎骨量减少者发生率为４４％（２２／５０）,ＯＰ为８％（４／５０）,高于对照组差别具有非常显著性意义（Ｐ〈０．０１）;股骨近端     

淋巴细胞凋亡在系统性红斑狼疮发病机制中的意义

系统性红斑狼疮是一种自身免疫病，它的主要特征是多种自身抗体的出现。本文从淋巴细胞凋亡角度对其发病机制进行了初步探讨，着重在淋收细胞凋亡与系统性红斑狼疮的关系，有效自身抗原的形成，自身抗体的产生机理，造成免疫性损伤的机制，系统性红斑狼疮与细胞因子，细胞凋亡机制的临床意义等方面阐述了淋巴细胞凋亡在系统性红斑狼疮发病机制中的重要地位。     

系统性红斑狼疮治疗新进展

本文简要综述了近几年在系统性红斑狼疮治疗中新型免疫抑制剂的应用以及各种生物治疗方法的进展。     

淋巴细胞凋亡在系统性红斑狼疮发病机制中的意义

系统性红斑狼疮是一种自身免疫病 ,它的主要特征是多种自身抗体的出现。本文从淋巴细胞凋亡角度对其发病机制进行了初步探讨 ,着重在淋巴细胞凋亡与系统性红斑狼疮的关系、有效自身抗原的形成、自身抗体的产生机理、造成免疫性损伤的机制、系统性红斑狼疮与细胞因子、细胞凋亡机制的临床意义等方面阐述了淋巴细胞凋亡在系统性红斑狼疮发病机制中的重要地位。     

系统性红斑狼疮骨关节肌肉表现

系统性红斑狼疮骨关节肌肉表现黄少弼肖征宇曾庆馀骨关节、肌肉是系统性红斑狼疮（ＳＬＥ）最常累及的组织，常被误诊为其它疾病。现将我院１９９０～１９９３年１００例ＳＬＥ骨关节、肌肉表现报告如下。临床资料一、一般情况１００例ＳＬＥ均符合１９８２年美国风湿病学...     

Neuropsychiatric systemic lupus erythematosus

Opinion statement The treatment of patients with neuropsychiatric systemic lupus erythematosus (NPSLE) can be difficult and complex owing to the variety of nervous system manifestations that can occur, which include peripheral nerve disease, headaches, seizures, cerebrovascular disease, chorea, transverse myelitis, and psychiatric and cognitive disorders. Many of these manifestations can result from metabolic abnormalities or infection or as side effects of medications. Thus, in any patient with suspected NPSLE, it is crucial to exclude secondary causes of the presenting symptoms before assuming that they are due to NPSLE. It is especially important to exclude infection because this is a common cause of both morbidity and mortality in patients with systemic lupus erythematosus (SLE). Symptoms such as anxiety and depression may or may not be related to disease activity. Treatment decisions are based on accurate diagnosis of the specific NPSLE manifestation, which is usually made using tools such as brain imaging, electroencephalography, cerebrospinal fluid analysis, nerve conduction studies, or special serologic tests (eg, determination of antiphospholipid or antiribosomal P antibody levels). It is also important to assess the degree of other SLEmediated systemic disease activity in a patient with neurologic manifestations to determine if activation of systemic disease activity is also occurring. This is done by measuring complement levels, anti-double-stranded DNA levels, complete blood count, and urinalysis. For some NPSLE manifestations (eg, infrequent seizures, headaches, depression, anxiety, or peripheral neuropathy) that appear without activation of systemic disease, symptomatic treatment is appropriate. For others (eg, psychosis, delirium, or transverse myelopathy without other obvious cause), treatment with high-dose glucocorticoids with or without cyclophosphamide is appropriate whether there is evidence of other systemic disease activity or not. In general, the activity and severity of the leading organ manifestations dictate pharmacologic treatment.     

Hypoparathyroidism in systemic lupus erythematosus

Hypoparathyroidism is a rare disease. The main cause of hypoparathyroidism is postsurgical hypoparathyroidism. However, cases of hypoparathyroidism in patients suffering from SLE exist although it is uncommon. Only three previous cases have been reported. We present the case of a woman suffering both from systemic lupus erythematosus and hypoparathyroidism. This reported association of hypoparathyroidism with lupus expands the spectrum of endocrine disorders seen in this disease. We suggest that there may be a common underlying pathophysiological process linking these diseases.     

Autoantibodies in systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a multifactorial disorder with multigenic inheritance and various environmental factors implicated in its aetiopathogenesis. Despite the multiple mechanisms involved in the aetiology of SLE being elusive, recent studies have made progress in our understanding of the pathogenic mechanisms via abnormal regulation of cell-mediated and humoral immunity that lead to tissue damage. The heterogeneity of the clinical manifestations probably reflects the complexity of the disease pathogenesis itself. The immune system in SLE is characterised by a complex interplay between overactive B cells, abnormally activated T cells and antigen-presenting cells. This interplay leads to the production of an array of inflammatory cytokines, apoptotic cells, diverse autoantibodies and immune complexes that in turn activate effector cells and the complement system, leading to tissue injury and damage which are the hallmarks of the clinical manifestations. SLE patients have dysregulation of inflammatory cytokines, chemokines and immune response-related genes, as well as of the genes involved in apoptosis, signal transduction and the cell cycle.     

Monogenic systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a rare autoimmune disease, which is more severe in case of pediatric onset. This may be due to greater involvement of genetic factors in comparison to adult forms. SLE is a multifactorial disease and is thought to be secondary to a combination of genetic, environmental factors as well as immunological defects. Studies on early-onset SLE and both familial and syndromic cases have led to the discovery of monogenic form of SLE, with autosomal or recessive inheritance. Related genes are responsible for complement deficiency and excessive production of interferon-alpha, both mechanisms studied in SLE pathogenesis. Apoptosis defect described in autoimmunity and lymphoproliferation syndromes is the human counterpart of lpr/lpr mouse model, deeply investigated as a murine SLE model. In this review, we discuss all monogenic Mendelian forms of SLE and underline the impact of this gene discovery on better understanding of SLE pathogenesis.     

The genetic contribution to systemic lupus erythematosus

We are currently witnessing an explosion of information about the genetic contribution to complex human diseases such as systemic lupus erythematosus (SLE). These genetic discoveries have profound implications for efforts to more fully characterize etiologic pathways and mechanisms. Further, these etiologic insights should lead to improved diagnostic and prognostic tools and inform the development of more specific therapies for SLE and related conditions. The article summarizes the evidence supporting a role for genetic factors in SLE, highlights the clinical and genetic complexity of the disease, reviews the genes that have established contributions to the risk of SLE and specific disease manifestations, and discusses the recent data emerging from genomewide association studies of SLE.     

Renal and systemic amyloidosis in systemic lupus erythematosus

A young male presented with oral ulceration for two years; swelling face and feet of seven days duration; diffuse goiter without signs of thyroid disease; normocytic normochromic anemia, thrombocytopenia, deranged renal functions, albuminuria of 2.5 g/24h with active urinary sediment. ANA and anti-ds DNA were positive, sonography of abdomen suggested medical renal disease. Testing for HIV, HBV, VDRL, CRP, rheumatoid factor, p-ANCA and c-ANCA were negative. Thyroid hormone assays were normal. Kidney biopsy done to stage lupus nephritis did not show any evidence of lupus involvement but staining for SAA amyloid was positive. Subsequent biopsies from the liver and rectum also stained positive for amyloid. Diagnosis of "Systemic lupus erythematosus with renal and systemic secondary amyloidosis with euthyroid diffuse goiter" was made. The case is being reported and discussed because of the interesting and rare association between amyloidosis and systemic lupus erythematosus.     

Genetic susceptibility to systemic lupus erythematosus in the genomic era

Our understanding of the genetic basis of systemic lupus erythematosus (SLE) has been rapidly advanced using large-scale, case-control, candidate gene studies as well as genome-wide association studies during the past 3 years. These techniques have identified more than 30 robust genetic associations with SLE including genetic variants of HLA and Fcγ receptor genes, IRF5, STAT4, PTPN22, TNFAIP3, BLK, BANK1, TNFSF4 and ITGAM. Most SLE-associated gene products participate in key pathogenic pathways, including Toll-like receptor and type I interferon signaling pathways, immune regulation pathways and those that control the clearance of immune complexes. Disease-associated loci that have not yet been demonstrated to have important functions in the immune system might provide new clues to the underlying molecular mechanisms that contribute to the pathogenesis or progression of SLE. Of note, genetic risk factors that are shared between SLE and other immune-related diseases highlight common pathways in the pathophysiology of these diseases, and might provide innovative molecular targets for therapeutic interventions.