系统性红斑狼疮并发蛛网膜下腔出血的临床特点

目的探讨系统性红斑狼疮(systemic lupus erythematosus,SLE)并发蛛网膜下腔出血(subarachnoid hemorrhage,SAH)患者的临床特点及预后。方法总结分析1983年1月至2012年1月就诊于北京协和医院的12例SLE并发SAH患者的临床资料。结果 SLE并发SAH患者住院期间的病死率高达42%(5/12)。SLE并发SAH的症状以头痛为主(75%)。系统性红斑狼疮疾病活动指数平均为(17.5±4.5)分。死亡患者在SAH发生早期即出现意识障碍者占80%(4/5),合并严重感染者占60%(3/5)。结论 SAH是SLE的少见且致命的并发症。对于出现意识障碍或合并感染的SLE患者,应当提高警惕,积极治疗SLE原发病及SAH,提高生存率。     

肝血管瘤合并系统性红斑狼疮2例报告

目的 总结肝血管瘤合并系统性红斑狼疮的诊治经验.方法 回顾性分析我院收治1例、文献报道1例肝血管瘤合并系统性红斑狼疮患者的临床资料,并探讨其病因学相关性.结果 系统性红斑狼疮理论上具有导致形成肝血管瘤的病因基础.此2例肝血管瘤合并系统性红斑狼疮患者起初均表现为难以解释症状的肝血管瘤.此2例患者均经过以糖皮质激素为主的治疗后好转.结论 临床工作者遇到难以解释症状的肝血管瘤时应警惕肝血管瘤并存系统性红斑狼疮的可能.肝血管瘤合并系统性红斑狼疮宜采用手术和糖皮质激素治疗.     

免疫与内分泌疾病

丁螺环酮在社交焦虑障碍治疗中的应用；影响强迫症治疗效果的相关因素探讨；超激光照射星状神经节治疗神经衰弱疗效观察；53例艾滋病患者机会性感染的治疗；系统性红斑狼疮脑病36例临床分析；血浆置换联合DNA免疫吸附血液灌流治疗难治性系统性红斑狼疮；来氟米特在狼疮性肾炎维持治疗中的疗效观察：中期研究报告[编者按]     

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目的探讨系统性红斑狼疮(SLE)合并急性胰腺炎的临床特点、病因、发病机制和治疗方法。方法回顾性分析1983-01～2003-03北京协和医院住院治疗的SLE合并急性胰腺炎患者15例。结果急性轻型胰腺炎12例,重型胰腺炎3例。所有患者均给予中到大剂量糖皮质激素治疗,病情痊愈5例,好转5例,死亡5例。结论(1)急性胰腺炎是SLE病情活动的表现;(2)胰腺血管病变是导致胰腺炎的主要致病机制;(3)糖皮质激素可能不是导致胰腺炎的病因;(4)中到大剂量糖皮质激素治疗是安全有效的。     

系统性红斑狼疮中认知功能障碍的研究进展

系统性红斑狼疮(SLE)患者中神经精神症状是很常见的,但病因和表现各不相同,其中认知功能障碍发生频率很高,不同的患者表现的程度也不同。近年来随着研究的深入,我们对SLE中认知功能障碍有了进一步的了解。1 SLE中认知功能障碍的概念与发生率SLE中神经精神症状临床表现多而复杂,1999年美国风湿病学会(ACR)给神经精神狼疮(NPSLE)定义,描述了SLE中19种神经精神症状,其中认知功能障碍是19种神经精神     

以消化系统症状为首发表现的系统性红斑狼疮15例临床分析

系统性红斑狼疮（SEE）是一种病因不明的慢性自身免疫性疾病,常累及全身多个系统,临床表现多样,早期症状多不典型,以消化系统症状为首发表现者常被诊断为消化系统疾病。现将近8年来我科收治的15例以消化系统症状为首发表现的系统性红斑狼疮患者的临床资料分析如下。     

系统性红斑狼疮心脏损害79例异常心电图分析

目的探讨系统性红斑狼疮患者心脏损害时的心电图表现,并分析年龄、性别、病程对心脏损害的影响。方法回顾性分析2006年6月至2012年6月中山市博爱医院诊断为系统性红斑狼疮的153例患者的临床资料,着重分析心电图资料。结果153例患者中79例(51.6%)具有心脏损害的异常心电图表现,其中窦性心动过速20例(25.3%),ST-T改变25例(31.6%)。9例患者具有心脏病相关症状(11.4%)。心脏损害组与无心脏损害组之间年龄、性别比较,差异无统计学意义(P>0.05);心脏损害组病程明显高于无心脏损害组,差异有统计学意义[22(9～43)个月vs.13(2～25)个月,Z=18.972,P<0.01]。结论系统性红斑狼疮可以累及心脏各个部分,其中以心电图窦性心动过速和ST-T缺血性改变最为常见;累及心脏时多属无症状型;心脏损害与病程有关。     

系统性红斑狼疮的消化系统表现及其诊治

系统性红斑狼疮(SLE)是一个多系统受累的自身免疫病.SLE消化系统症状发生率为25%～50%,约10%SLE患者以消化系表现为首发症状[1].SLE消化系统累及发生率高,不具特异性,并且与感染、药物、血栓形成等原因引起的症状鉴别困难,易误诊,尤其是以消化系统症状为首发表现的SLE更易误诊[2].     

系统性红斑狼疮合并中枢神经系统感染30例临床分析

目的 探讨系统性红斑狼疮(SEE)合并中枢神经系统感染病例的临床表现、治疗及预后特点. 方法 回顾性分析30例诊断明确,资料完整的SLE合并中枢神经系统感染患者.结果 1986年1月至2007年3月共收治SLE患者3039例,其中合并中枢神经系统感染者30例占1%,平均年龄(34±11)岁,其中女性27例,男性3例.30例患者中结核性感染11例占37%,非结核的细菌感染11例占37%,真菌感染8例占26%.中枢神经系统感染的患者以发热、头痛、意识障碍为常见临床表现.预后方面3组间差异无统计学意义. 结论 SLE患者合并中枢神经系统感染表现不典型,最常见的是结核性脑膜炎,及早行腰椎穿刺检查有助于早诊断.低自蛋白血症、低C3及低颅压提示预后不佳.     

不完全系统性红斑狼疮患者的随访研究及病情进展预测

不完全系统性红斑狼疮(ILE)是指患者具有系统性红斑狼疮(SLE)样临床表现及血清学特点,但是并不能完全符合美国风湿病学会(ACR)修订的SLE的分类标准,这种患者也被称为隐匿型狼疮。为了进一步了解ILE的病情进展及相关危险因素,我们对19例中国汉族ILE患者进行了为期3年的随访研究。对象与方法1.对象:19例ILE患者均为本院门诊患者。入选标准:(1)符合2~3条ACR1997年SLE分类标准;(2)不能符合其它弥漫性结缔组织病的分类标准;(3)无其它结缔组织病的特征性临床表现,如侵蚀性关节炎、皮肤硬化等。19例ILE患者中18例为女性,1例为男性。年…     

Cutaneous lupus erythematosus: Diagnosis and treatment

Cutaneous lupus erythematosus (CLE) encompasses a wide range of dermatologic manifestations, which may or may not be associated with the development of systemic disease. Cutaneous lupus is divided into several sub-types, including acute CLE (ACLE), sub-acute CLE (SCLE) and chronic CLE (CCLE). CCLE includes discoid lupus erythematosus (DLE), LE profundus (LEP), chilblain cutaneous lupus and lupus tumidus. The diagnosis of these diseases requires proper classification of the sub-type, through a combination of physical examination, laboratory studies, histology, antibody serology and occasionally direct immunofluorescence, while ensuring to exclude systemic disease. The treatment of cutaneous lupus consists of patient education on proper sun protection along with appropriate topical and systemic agents. Systemic agents are indicated in cases of widespread, scarring or treatment-refractory disease. In this chapter, we discuss issues in classification and diagnosis of the various sub-types of CLE, as well as provide an update on therapeutic management.     

Drug-induced lupus erythematosus secondary to nafcillin: the first reported case

With an estimated incidence of 15–30,000 cases per year in the United States, drug-induced lupus erythematosus (DIL) is an uncommon iatrogenic condition. The number of implicated medications increases each year. We report the first case of DIL secondary to nafcillin administration in a patient with a prosthetic aortic valve and methicillin-sensitive Staphylococcus aureus who developed a facial rash and lower back pain.The opinions expressed herein are those of the authors and do not reflect official opinion of the US Department of the Navy or US Department of Defense.     

Comparación de datos demográficos,presentación clínica,tratamiento y desenlace de pacientes con lupus eritematoso sistémico tratados en un centro público y otro privado de salud en Santa Fe,Argentina

The study includes 159 SLE patients seen between 1987 and 2011, of whom 116 were treated in the public health system and 43 in private practice. In the comparison between both groups, it was shown that patients in the public health system were younger at first consultation and at the onset of SLE, and that the mean duration of their disease prior to nephropathy was statistically significantly shorter. They also presented with more SLE activity (measured by Systemic Lupus Erythematosus Activity Index) such as fever, lower levels of C4, and elevated erythrocyte sedimentation rate. Although cyclophosphamide was administered more frequently to patients in the public health system group, there were no statistically significant differences in renal histological findings. A second renal biopsy was performed on 20 patients due to the presence of persistent proteinuria, peripheral edema, urinary casts, or because of previous defective renal specimens. The overall 10-year survival of the patients in the public health system was 78% compared to a survival rate of 91% for the patients in private practices. When survival was evaluated at 15 years, however, no differences were found (log rank test: 0.65). Patients from both public and private groups attended medical specialist practices and received early diagnoses and close follow-ups.     

Lupus erythematosus tumidus (LET) with autoimmune thyroid dysfunction (AITD) as the first presentation of systemic lupus erythematosus: A case report and review of the literature

IntroductionLupus erythematosus tumidus (LET) is a rare cutaneous manifestation especially as a first presentation of systemic lupus erythematosus (SLE). Autoimmune thyroid dysfunction (AITD) may be associated with SLE but rarely at initial presentation, and its diagnosis may be delayed.Case reportA 29 year old male presented to Tishreen Hospital in Damascus with a three-year history of recurrent cellulitis-like lesions on the face, and more recently, he developed similar lesions on the trunk and the chest, in addition to the development of peripheral and scrotal edema, constipation, xeroderma, hair loss, musculoskeletal pain and depression. Laboratory investigations revealed: leukopenia, anaemia, elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Immunological tests identified the positive anti-nuclear antibody (ANA), anti-double stranded DNA (anti-dsDNA), anti Ro/SSA, anti La/SSB antibodies. Additionally, there was consumed complement C3, elevated thyroid stimulating hormone (TSH), thyroid hormones decreased free T3 and T4 and anti-thyroid peroxidase (anti-TPO) antibody was positive. Skin biopsy from the cheek plaque suggested the presence of LET and revealed slight hyperkeratosis; actinic elastosis, telangiectasia and edema of the papillary dermis; deep dermis perivascular and periadnexal inflammatory infiltrates with karyorrhexis of the lymphocytes and dermis edema between strands of collagen. The patient fulfilled the SLE classification criteria and consequently, methylprednisolone, azathioprine, hydroxychloroquine, levothyroxine were introduced with dramatic improvement.ConclusionLET is a rare cutaneous lupus-specific lesion that may be associated with SLE. AITD, hypothyroidism in particular, could be an initial presentation of SLE. Increased awareness and early diagnosis of such clinical presentations may improve patient outcomes.     

Lupus in the far east: a modern epidemic

Systemic lupus erythematosus (SLE) is a diverse and heterogeneous disease with much variation between different ethnicities. Although more severe and prevalent in non‐Caucasian populations, the bulk of evidence regarding ‘Asian lupus’ had traditionally been extrapolated from studies on patient minorities in the West. Lupus research in Asia has since grown exponentially and now takes a leading role in improving the care for SLE patients worldwide. With recent advances, particularly from studies in population‐specific phenotyping, genome‐wide association studies, lupus nephritis and innovative treatment modalities, we now have an expanding understanding of both the basic science and clinical management of SLE. Upcoming breakthroughs from growing multi‐ethnic cohorts and international collaborations will likely bring further important ramifications for SLE management in the near future.     

Response to hydroxychloroquine in Japanese patients with systemic lupus erythematosus using the cutaneous lupus erythematosus disease area and severity index (CLASI)

Abstract

We evaluated the cutaneous lupus erythematosus disease area and severity index (CLASI) in Japanese patients with systemic lupus erythematosus (SLE) in order to design a clinical trial of hydroxychloroquine (HCQ) in Japan. Our prospective cohort study consisted of seven SLE patients with active skin disease who started HCQ at Tokyo Metropolitan Tama Medical Center. The therapeutic responses were assessed at 4 months. Patients were categorized as responders (improved) or non-responders (unchanged or worsened) using the criteria of a 4-point or 20% decrease in the CLASI activity score. We also assessed joint pain determined by patient visual analog scale (VAS), malaise (VAS), patient global assessment of SLE (VAS), and constitutional and musculoskeletal symptoms according to the British Isles Lupus Assessment Group (BILAG) disease activity index. Six patients (86%) were categorized as responders. The median (range) CLASI activity score of all patients at assessment had changed from 8.0 (2–22) to 4 (2–10). All five patients with joint pain and all five patients with malaise showed improvement in patient VAS but the BILAG findings failed to capture these improvements. In conclusion, the cutaneous aspects of SLE can be measured by the CLASI. The CLASI activity score may be a reasonable primary endpoint when performing a clinical trial of HCQ.     

Response to hydroxychloroquine in Japanese patients with lupus-related skin disease using the cutaneous lupus erythematosus disease area and severity index (CLASI)

Background

Hydroxychloroquine (HCQ) is not available in Japan. To design a clinical trial of HCQ, we evaluated the response to HCQ in Japanese patients with lupus-related skin disease using the cutaneous lupus erythematosus disease area and severity index (CLASI).

Methods

Twenty-seven patients with lupus-related skin disease who started HCQ at four hospitals were included. Patients were categorized into responders by the CLASI response criteria. The points and the rate of improvement in the CLASI activity score after 16 weeks of treatment were analyzed, focusing on six parameters: systemic lupus erythematosus (SLE), skin manifestations, disease duration, prednisolone, smoking, and severity.

Results

Twenty-seven patients, including 17 with SLE (6 with SLE/Sjögren’s syndrome), were analyzed retrospectively. Twenty-three patients (85 %) were categorized as responders. The mean CLASI activity score improved from 10.1 to 4.5 (p < 0.0001). The improvement rate did not differ in these parameters except for that of annular erythema (81.6 versus 34.3 %, p = 0.036). On multivariate analysis, the baseline CLASI activity score (CLASI ≥9) correlated with the greatest decrease in CLASI activity score (F = 69.7, p < 0.0001).

Conclusions

CLASI is a reliable indicator to evaluate the efficacy of the drug, and HCQ is an effective treatment for Japanese patients with lupus-related skin disease.     

Antinuclear antibodies measured by enzyme immunoassay in patients with systemic lupus erythematosus: relation to disease activity

To evaluate the correlation between measurements of antinuclear antibodies serum levels by enzyme immunoassay (ANA-EIA), and the degree of systemic lupus erythematosus disease activity. To retest the performance of the test compared to measurement of antinuclear antibodies by immunofluorescence (ANA-IIF). Eighty-five sera from 71 patients with SLE were tested. Demographic, clinical, laboratory, and SLEDAI status were collected. The sera were tested for ANA-EIA and by ANA-IIF at 1:40 and 1:160 dilutions. Serum levels of ANA-EIA were compared to the overall SLEDAI score and to each of its components. A SLEDAI score of ≥6 was considered clinically significant. The sera of fifty-one healthy volunteers served as controls. Serum levels of ANA-EIA were significantly higher in patients with a SLEDAI score of ≥6 compared to the group of patients with a SLEDAI score of <6 (P = 0.004). High serum levels of ANA-EIA correlated significantly with elevated anti DS-DNA antibodies (P < 0.001), low C₃ or C₄ levels (P < 0.001), pyuria (P < 0.011), arthritis (P = 0.019), and new rash (P = 0.019). Levels of ANA-EIA were significantly higher in patients tested positive by IIF compared to those who tested negative. Higher serum levels of ANA-EIA correlated with clinically significant disease activity in patients with SLE. Higher serum levels of ANA-EIA also correlated with some single items of the SLEDAI. The results also reiterated the validity of ANA-EIA testing in patients with SLE. Further longitudinal studies are needed in order to test the hypothesis that serum ANA-EIA levels might reflect fluctuations in disease activity.     

Recurrent fractures in an elderly patient with systemic lupus erythematosus

Glucocorticoid‐induced osteoporosis (GIO) is an important problem that remains undertreated, even by rheumatologists. We present a case of an elderly patient with systemic lupus erythematosus diagnosed more than 40 years ago, who suffered from recurrent fractures and attendant complications despite a bone mineral density (BMD) score in the osteopenic range and treatment with bisphosphonates. With improved treatment and outcome of lupus, an increasing number of elderly patients who are susceptible to osteoporotic fractures are expected. This case serves to highlight that rheumatic disease patients on steroids should be screened for GIO, as effective treatment and preventive measures are available. Teriparatide is a promising treatment for patients who have failed bisphosphonate treatment or who are at high risk for fracture. We should also bear in mind that BMD scores alone are not indicative of fracture risk, and other tools such as the WHO‐FRAX (Fracture risk assessment tool), serum vitamin D3 levels and bone turnover markers should be used where appropriate. Other measures including attention to factors that contribute to falls should also be considered, necessitating a multi‐disciplinary approach.