老年系统性红斑狼疮的诊治特点

1　老年系统性红斑狼疮发病特点系统性红斑狼疮 (ｓｙｓｔｅｍｉｃｌｕｐｕｓｅｒｙｔｈｅｍａｔｏｓｕｓ ,ＳＬＥ)是以多系统受损为特点的自身免疫性疾病。据我国流行病学的初步调查 ,患病率为70·10 - 5～ 10 0·10 - 5。老年ＳＬＥ一般是指首次发病年龄≥ 5 0岁者 ,其发病率约占ＳＬＥ的 6 %～ 12 %。与中青年发病的ＳＬＥ一样 ,以女性多见。 6 5岁以上者男女比例缩小 ,接近 1∶2～ 3。近年 ,老年ＳＬＥ患者有增加趋势 ,一是由于社会老龄化 ,二是对老年ＳＬＥ的诊断经验的不断积累使误诊率下降 ,三是治疗方法的不断改进使患者的存活率增…     

Preclinical and early systemic lupus erythematosus

The challenge of early diagnosis and treatment is a timely issue in the management of systemic lupus erythematosus (SLE), as autoimmunity starts earlier than its clinical manifestations. Hence, growing efforts for stratification of patients according to the individual risk of developing specific clinical manifestations and/or predicting a better response to a given treatment have led to the proposal of several biomarkers, which require validation for use in clinical practice. In this viewpoint, we aim at distinguishing and discussing the features and the approach to asymptomatic immunological abnormalities potentially heralding the development of SLE, defined as preclinical lupus, and clinical manifestations consistent with SLE not yet fulfilling classification criteria, defined as early lupus. In case of preclinical SLE, careful surveillance using available screening tools is paramount, while patients with early lupus deserve an appropriate and timely diagnosis and, consequently, a proper treatment including hydroxychloroquine as the anchor drug.     

Adherence to treatment in systemic lupus erythematosus patients

Adherence is defined as “the extent to which a person's behaviour coincides with medical or health advice.” Poor adherence to therapeutic regimens is a common and expensive problem in patients with chronic diseases including systemic lupus erythematosus (SLE) and is associated with a higher risk of flares, morbidity, hospitalisations and poor renal outcome. Non-adherence to the treatment is multifactorial for most patients and varies according to unintentional or intentional patterns. The rates of non-adherence in SLE patients range from 3% to 76% depending on the assessment methods, which are all subject to limitations. Indeed, poor adherence to therapeutic regimens is difficult to evaluate. Two studies have shown that undetectable blood hydroxychloroquine (HCQ) concentration may be a simple, objective and reliable marker of non-adherence in SLE patients. The accurate diagnosis of non-adherence may prevent one from incorrectly interpreting disease manifestations as a lack of response. It may then avoid an unnecessary or even dangerous treatment escalation.     

Bromocriptine treatment of systemic lupus erythematosus.

Prolactin, a peptide hormone, acts as a cytokine. It has been hypothesized that bromocriptine, a dopamine analog that suppresses pituitary secretion of prolactin, suppresses circulating prolactin and, through this mechanism, has the potential to suppress autoimmune disease. This rationale has been applied to the treatment of systemic lupus erythematosus (SLE), a prototype autoimmune illness that occurs spontaneously in animal models such as the F1 hybrid NZBxNZW mouse, and in humans. Treatment with bromocriptine was effective in treating some induced and spontaneous autoimmune disease in experimental models. Bromocriptine did slow the course of SLE in NZBxNZW mice when treatment was started before the appearance of clinical disease. In addition, bromocriptine was effective in treating established disease in this model. In three separate clinical trials, bromocriptine showed evidence that it had a therapeutic effect in treating human lupus. Bromocriptine is currently considered an unproven therapy for SLE. Its use is entirely experimental. The fact that bromocriptine was effective in treating NZBxNZW mice, the beneficial therapeutic effects in human trials, and the low toxicity of the drug form a solid rationale for undertaking further therapeutic trials.     

Lupus erythematosus tumidus in systemic lupus erythematosus: novel association and possible role of early treatment in prevention of discoid lupus erythematosus

Skin involvement in systemic lupus erythematosus (SLE) occurs in varied forms. Lupus erythematosus tumidus (LET) is not known to occur with SLE. In our patient presented below, LET occurred in a patient with SLE. Some of the lesions progressed to scarring discoid lesions, while others responded very well to hydoxychloroquine treatment. We, as rheumatologists, are not very aware of this entity, which is so amenable to prevention and treatment if recognized correctly.     

系统性红斑狼疮并发感染的危险因素临床特点及早期诊断

感染是影响系统性红斑狼疮(SLE)患者病死率的重要因素之一,故早期诊断感染并发症能显著改善SLE患者预后。血清C-反应蛋白(CRP)、降钙素原(PCT)有助于鉴别SLE并发感染和SLE活动,血清KL-6有助于鉴别SLE伴发感染和SLE累及肺脏。SLE合并感染的病原体除了最多见的细菌感染外,病毒和真菌感染近几年有上升趋势,需要引起足够重视。     

Pulmonary involvement in early systemic lupus erythematosus

Aim of the workSLE is an autoimmune disease characterized by a variety of clinical and laboratory abnormalities. It may affect many organs and pulmonary involvement is a common finding in SLE. The purpose of this study was to disclose the pulmonary involvement in early SLE patients not more than 2 years of disease duration using the computed tomography (CT) as well as the pulmonary function tests as ways of pulmonary involvement assessment.Patients and methodsForty-two patients aged 29 ± 12.5 with early SLE not more than 2 years of disease duration were recruited for the study. All patients were assessed clinically for their SLE with BILAG which was utilized for disease activity determination.ResultsNine and half percent of our patients were found to be clinically involved by ILD, where 28.6% have abnormal HRCT finding, 26.2% with abnormal PFT. Variants that were associated with an abnormal forced vital capacity FVC < 80% in a significant manner were: smoking, long disease duration, self-reported pulmonary symptoms (p 0.001), BILAG global score (p 0.006), Anti dsDNA (p 0.001), Antiphospholipid (IgM or IgG) (p 0.01), anti Sm (p 0.002), anti-RNP (p 0.005), HRCT abnormalities (p 0.001), current medication of steroid (any dose) (p 0.005), immunomodulator therapy (p 0.002), and Rituximab therapy (p 0.001).ConclusionsILD occurs as early as in the first 2 years in the course of SLE patients. There was a clear predilection of ILD with certain variables in our cohort of patients.     

Lessons learned from anti-CD40L treatment in systemic lupus erythematosus patients

The CD40-CD40L system has pleiotropic effects in a variety of cells and biological processes including immune response, thrombosis and atherogenesis. Within the immune system, these molecules represent a critical link between its humoral and cellular arms. As a result of these attributes and based on preclinical data in animals, anti-CD40L antibodies were tested in a variety of immunologic diseases including idiopathic thrombocytopenic purpura, psoriasis, Crohn's disease, systemic lupus erythematosus and transplantation. Phase I/II studies in humans with lupus nephritis demonstrated reduction of anti-double-stranded DNA (anti-dsDNA) antibodies but not of protective antibodies. Reduction of anti-DNA was associated with increased serum complement levels and reduced glomerular inflammation. As a result of thrombotic effects, observed even in patients negative for anti-cardiolipin antibodies, there is a temporary halt on further human studies. The reasons for the prothrombotic effects are not clear at present but may represent effects on platelets and/or the endothelium. In view of the significant immunomodulatory effects of anti-CD40L treatment in patients with lupus nephritis, the increasing realization of the importance of premature atherosclerosis in lupus and an increasing amount of data supporting a role for the CD40-CD40L interactions in this process, inhibition of this pathway deserves further exploration in lupus.     

Tuberculosis in patients with systemic lupus erythematosus.

Tuberculosis associated with systemic lupus erythematosus (SLE) was studied in a cohort of 311 patients seen between 1963 to 1979. There were 16 such patients, giving rise to a prevalence rate of 5%. The characteristics of SLE-associated tuberculosis include a high incidence of miliary and far-advanced pulmonary disease, delay in establishing diagnosis, especially the extrapulmonary form, and tendency to attribute symptoms like fever, malaise, and weight loss to the lupus process. Treatment was successful in 9 patients. Of the 7 death 5 were attributed directly to the mycobacterial infection and 2 to complications of SLE.     

99mTcDTPA-aerosol clearance in early diagnosis of pulmonary changes in patients with active systemic lupus erythematosus

Nineteen patients with active SLE and a control group of 20 healthy persons were investigated. We have made to all of them 99mTcDTPA-aerosol scintigraphy and the following pulmonary functional tests--Dc CO, Dm, Vc, HRCT. Accelerated clearance of 99mTcDTPA was found in all patients with active SLE. The half-elimination time T1/2 was shortened and varied between 22.6 +/- 4.7 min for the right one. It wasn't established correlative dependence between 99mTcDTPA and the pulmonary functional tests. In 36.8% of the patients HRCT was normal, while 99mTcDTPA clearance was accelerated. This fact shows, that 99mTcDTPA-aerozsol scintigraphy is more sensitive method for early diagnosis of pulmonary changes, than the pulmonary functional tests and HRCT.     

'Concealing the evidence': the importance of appearance concerns for patients with systemic lupus erythematosus

Outwardly visible signs associated with systemic lupus erythematosus (SLE) can include facial rashes, alopecia and weight gain. We sought to understand the concerns of SLE patients about their appearance and the recognition of this by healthcare professionals. Semi-structured interviews were carried out with 10 women aged 26-68 years diagnosed with SLE for one to 12 years. Data were analysed with Interpretative Phenomenological Analysis (IPA); this seeks to describe and provide understanding of people's experience of a phenomenon by studying in-depth a small number from a relatively homogeneous group (women with SLE in the present study). Analysis revealed three themes concerning appearance issues. Participants described public self-consciousness after the onset of SLE. Cosmetics and clothing were used skilfully to appear 'normal', hide the 'self' and assert control but could increase feelings of difference and isolation. Self-imposed isolation was also described and may relate to depression. The understanding of family, friends, colleagues and healthcare providers was also important. Awareness of the psychosocial concerns of SLE patients with life-changing skin disease may enable multidisciplinary healthcare teams to offer a more sensitive, practical service. The physical and emotional needs of SLE patients need to be ascertained and appropriate educational and psychological services are required.     

系统性红斑狼疮的心脏损害

目的：探讨系统性红斑狼疮（SLE）的心脏受累表现，并分析年龄、性别，病程及抗心磷脂抗体（ACL）与SLE心脏损害的相关性。方法：回顾性总结分析1998年9月至2000年8月我院诊断SLE的入院病人272例，结果：272例SLE病人中145例（53．3％）具有心脏损害，10例病人具有心脏病相关症状（6．7％），心脏损害包括心电图（ECG）ST－T异常61例（42．1％），心包积液60例（41．4％），肺动脉高压23例（15．9％），心肌损害22例（15．2％），心律失常20例（13．8％），瓣膜病19例（13．1％），左心耳血栓1例（0．6％）。心脏损害组与无心脏损害组之间年龄，性别差异无差异性（P＞0．05，病程差异有显著性（P＜0．01），ACL阳性组与阴性组瓣膜损害发生率差异有显著性（P＜0．01），结论：SLE可以累及心脏各个部分，其中以ECC ST－T缺血性改变和心包受累最为常见。SLE累及心脏时多属无症状型，SLE心脏损害与病程有关，抗心磷脂抗休与心脏瓣膜损害关系密切。     

Infection in systemic lupus erythematosus patients

BackgroundInfection is a leading cause of morbidity, mortality and hospital admission in systemic lupus erythematosus (SLE) patients.Aim of the workTo study infection in SLE patients regarding site of infection, pathogenic organism, hospitalization and/or intensive care unit (ICU) admission.Patients and methodsThis study included 79 patients. SLE disease activity index (SLEDAI-2K) and damage index were evaluated. Detailed information about the site of infection and pathogens were reported.Results71 females and 8 male patients (F:M 8.9:1), with a mean age of 29 ± 9.6 years (17–55 years) and disease duration of 5.9 ± 5.7 years, 55 (69.6%) patients had infection at time of study while 24 (30.4%) did not. The SLEDAI-2 k and damage index were significantly higher in SLE patients with infection (14.2 ± 11.8 and 3.7 ± 3.7) compared to those without infection (5.9 ± 5.03 and 1.8 ± 1.3) (p = 0.03 and p = 0.045 respectively). Those with infection had a shorter disease duration (4.9 ± 5.2 vs 8.3 ± 6.2; p = 0.005), received more cyclophosphamide (56.4% vs 16.7%; p = 0.001), higher erythrocyte sedimentation rate (ESR) (75.5 ± 27.1 vs 35.8 ± 24.7 mm/1^sthr) (p < 0.0001) and consumed complement (C3) (71.1 ± 28.4 vs 97.2 ± 28.2; p < 0.0001). 17/55 (30.9%) had more than one site of infection and 46/55 (83.6%) required hospital admission. 17 (30.9%) of hospitalized patients were transferred to the ICU. The main pathogenic organisms were bacterial (40%), fungal (27.3%), viral (10.9%) and unconfirmed in 21.8%. Chest was the commonest site (40%) followed by the skin (34.4%), oropharynx (25.5%) and urinary tract (20%).ConclusionInfection is an important cause of hospital and ICU admission in SLE patients. Early disease, disease activity and damage, cyclophosphamide, ESR and consumed C3 were associated with infection in SLE.     

Osteoporosis in systemic lupus erythematosus: prevention and treatment

The patient with systemic lupus erythematosus (SLE) is at risk of osteoporosis through several factors: the inflammatory disease itself, disease-related co-morbidity, and its treatment. Bone loss is apparent early in the disease and this may be confounded primarily by treatment with corticosteroids. Patients should be assessed for additional risk factors for osteoporosis and general lifestyle measures adopted. Bone mineral density measurement should be considered in SLE patients at high risk of osteoporosis, particularly those starting corticosteroids and in postmenopausal women. Calcium and vitamin D supplementation provide general prophylaxis and are a suitable first-line option. Hormone replacement should be used in hypogondal subjects unless contra-indicated. In subjects at high fracture risk, particularly in postmenopausal women, bisphosphonate therapy should be considered as these agents have been shown to significantly reduce vertebral fracture risk. These measures should reduce the burden of osteoporosis and fracture in patients with lupus.