影响系统性红斑狼疮活动指数评分的主要指标分析

目的 探讨系统性红斑狼疮(SLE)住院患者的分布特征、临床表现、实验室指标及活动指标,为临床诊断提供更多的参考.方法 收集2006年1月至2010年6月宁夏医科大学总医院1037例SLE住院患者的病案资料.采用t检验和x2检验进行统计学分析.结果 5年住院病例呈逐年上升趋势;发病特点以20～40岁女性最为常见,占67.5％,男女比例为1∶8.26.关节痛是最常见的首发症状,占54.3％,其次是皮疹,占48.2％;补体C3下降、蛋白尿、血小板降低和抗dsDNA阳性可以作为SLE早期诊断的指标.综合分析SLE疾病活动指数(SLEDAI )＞9的患者占26.0％,影响SLEDAI活动指数评分的主要指标是:发热、关节痛、皮疹、蛋白尿、补体降低、抗dsDNA抗体滴度升高、胸膜炎、脱发、口腔溃疡、心包炎、活动性神经精神症状、血小板降低;活动组在发热、关节痛、皮疹、面部红斑、肺脏损害、脱发、口腔溃疡、心脏损害、神经精神症状和肾脏损害等的发生率均高于非活动组.抗dsDNA抗体滴度、红细胞沉降率和C3、C4水平异常发生率等指标在活动组均高于非活动组.结论 SLE是多系统、多脏器受累的疾病,在临床症状和免疫学指标上有其特征性改变,因而重视早期诊断,掌握疾病的发病特征和规律,正确判断病情的活动,对采取合理有效的治疗,延缓SLE患者病情发展,改善预后具有重要意义.     

系统性红斑狼疮男性患者临床及免疫学特征

目的 分析142例男性系统性红斑狼疮(SLE)患者起病时首发及发病过程中临床表现特征、自身抗体分布以及其他实验室指标改变特征,探讨SLE男性患者的临床及免疫学特征.方法 收集1994年4月至2010年4月于本院风湿免疫科住院的男性SLE患者142例,年龄12 ～ 68岁,平均年龄31.9岁;同期住院女性SLE患者320...     

类风湿关节炎患者就医及治疗现状分析

目的 调查和分析类风湿关节炎(RA)患者就医行为特点及规范化治疗状况.方法 对北京大学人民医院2007年5月至8月门诊就诊的181例RA患者进行现场调查.内容包括患者的一般资料、就诊及确诊时间、科室,随诊时间间隔以及改善病情抗风湿药(DMARDs)的应用情况.结果 本组患者平均年龄为(53±11)岁,男女之比为1:4.2,病史为(10±8)年.患者首次就诊科室以骨科为最多(32.0%,58/181),其次为综合内科(24.9%,45/181),风湿免疫科(23.2%,42/181).而确诊科室则以风湿免疫科占大多数(62.4%,113/181),其次为综合内科(16.0%o,29/181),骨科(8.3%,15/181).从患者出现症状到确诊RA的中位时间为6个月(四分位间距2～15.8个月).随诊时间间隔较长的患者所占比例较高,其中间隔在1年以上者达19.6%.间隔在0.5～1年者占24.4%.56.2%(45/80)的RA患者就诊前未接受规范化治疗.甲氨蝶呤是患者应用最多的DMARDs,占67.3%,其次为来氟米特(46.4%)、柳氮磺吡啶(37.5%)及羟氯喹((19.6%).结论 患者在风湿免疫专科首次就诊比例低,就诊及确诊均存在时间延误,半数以上的患者未接受正规的DMARDs治疗.     

儿童风湿性疾病561例回顾性分析

目的 了解河南地区儿童风湿性疾病(JRD)的患病情况、地区分布、各种JRD的构成比、首发症状、主要症状和实验室检查等临床特点，增加对JRD的认识。方法 对1990年1月至2003年8月在河南省人民医院儿科住院的561例JRD患者进行回顾性分析。结果 过敏性紫癜(HSP)、幼年特发性关节炎(JIA)、风湿热(RF)、儿童系统性红斑狼疮(SLE)农村患者均多于城市，川崎病(MCLS)城市患者较多，儿童风湿性疾病的首发症状以关节痛、皮疹、发热最多见，主要症状以皮疹、关节痛和发热最多见，主要累及器官为肾脏、淋巴结、心脏和肝脏。43.1％的JRD患者白细胞升高，61.6％的患者血沉(ESR)升高，类风湿因子阳性以JIA最多见，本组病例死亡3例。结论 JRD在儿科住院中的百分比为2.9％,最常见的病种为HSP、JIA、MCLS、RF、SLE，HSP发病有升高的趋势，RF发病呈下降趋势，需加强对JRD患者的认识，提高诊断和治疗水平是控制病情、减少并发症的关键。     

误诊为肺间质纤维化的显微镜下多血管炎1例并文献复习

抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎常常表现为多系统损害,以肾脏和肺的受累最为常见,亦常有不规则发热、疲乏、皮疹、关节痛、肌痛、腹痛、神经炎和体质量下降等非特异性症状.由于临床表现复杂,常易误诊、漏诊.我们近期在病房诊断1例首发首诊为特发性肺间质纤维化的显微镜下多血管炎(MPA).     

以消化系统症状为首发表现的系统性红斑狼疮临床特征分析

目的 分析以消化系统症状为首发表现的系统性红斑狼疮(SLE)患者的临床特征.方法 采用回顾性方法 ,选取2004年7月至2009年6月因不明原因腹痛、腹泻等消化系统症状入消化科住院并最后确诊为SLE的患者为研究对象(研究组),并按1:2比例选取以发热、关节痛等为主诉的风湿免疫科同期住院SLE患者作为对照组,对临床特征进行比较分析.结果 共收集到研究组31例(其中女性27例)、对照组62例(其中女性55例).经多因素分析后发现,研究组以腹痛(OR=7.91)和腹泻(OR=6.26)症状最为常见,确诊之前的病程较对照组长(OR=1.41),体重下降较对照组明显(OR=1.37),但红细胞沉降率(ESR)增快(OR=0.88)和尿红细胞阳性(OR=0.56)不如对照组明显.结论 临床应注意识别以消化系统症状为首发表现的SLE患者,以减少误诊和漏诊.     

狼疮性肾炎的临床表现

LN临床上表现为肾脏损害和全身损害两方面。一、肾脏损害的临床表现:临床上具有肾脏病表现的约占70%。大多数LN患者首先发现肾外的多系统损害。诊断SLE后1～3年内出现肾脏病的临床表现,也有个别患者延迟至15年后方产生肾脏病的改变。少数患者在疾病早期并无多系统损害,血清学检查亦不支持SLE的诊断,仅表现为尿或肾功能异常,     

系统性红斑狼疮患者血液学指标相关性43例分析

目的研究系统性红斑狼疮(SLE)患者的血象及骨髓特点.方法对43例SLE患者的血象及骨髓象进行涂片染色镜检并进行临床观察.结果SLE临床表现以贫血为主(86%),另有不同程度的发热(占53.5%),面部红斑(占65.1%),部分患者并发有其他症状.SLE患者血象WBC、Hb及PLT均有不同程度减少;骨髓象增生活跃程度不一,部分患者可出现原始细胞、早幼粒细胞及嗜酸性细胞增多.红系80%以上增生良好,但也有部分患者表现为缺铁性贫血、溶血性贫血及混合性贫血.巨核细胞增生正常的占65.1%,另有少数增生减低或缺如.结论SLE临床表现不一,多以贫血、血小板减少等为首发症状,部分患者骨髓象可出现原始细胞及早幼粒细胞增多.     

成人过敏性紫癜35例临床分析

目的 分析成人过敏性紫癜(HSP)临床特点,以提高临床诊治水平.方法 对1993年9月至2005年12月天津医科大学总医院血液科收治的35例成人HSP患者的发病特点、临床表现、相关实验室检查等方面资料进行回顾性分析.结果 成人HSP以男性及青壮年患者为主.冬季为主要发病季节.上呼吸道感染及进食鱼、虾、蟹等为主要诱因.多数患者以皮肤损害为首发表现.混合型HSP多见.所有患者在病程中均先后出现皮肤损害,双下肢为皮疹最易侵犯的部位.有关节损害者15例(42.9%).有胃肠道损害者20例(57.1%).有肾脏损害者20例(57.1%).结论 成人过敏性紫癜的临床特点有其特殊性,易发生严重肾损害和多器官受累.     

系统性红斑狼疮患者心脏受累的心电图改变

目的探讨系统性红斑狼疮(systemic lupus erythematosus,SLE)心脏损害心电图检查的临床意义。方法收集2007年4月至2008年1月入住我院风湿免疫科的60例SLE患者的心电图资料和超声心动图检查结果,进行回顾性分析。结果 60例确诊SLE患者,没有心脏受累症状者52例,但心电图检查发现31例有心电图改变,阳性率51.7%,47例患者进行了心脏超声检查,发现39例心脏结构或功能改变,阳性率83.0%。结论 SLE患者常规心电图检查,可以发现无症状的心脏损害患者的心电图改变,进一步做心脏超声等检查,以明确红斑狼疮所致的心脏损害的程度,为治疗提供重要的早期诊断方法。     

Dengue fever evolving into systemic lupus erythematosus and lupus nephritis: a case report

Dengue viremia may be the trigger for immune complex formation in patients who are predisposed to developing autoimmune disease. We report a rare case of dengue virus infection evolving into systemic lupus erythematosus (SLE) and lupus nephritis. To the best of our knowledge this is the first case of dengue fever evolving into lupus nephritis. A 22 year old female presented with having had high grade fever, skin rash, breathlessness, retro-orbital pain, abdominal pain, arthralgias and myalgias for 10 days. She tested positive for dengue immunoglobulin M (IgM). She was given supportive treatment and was subsequently discharged. Four weeks later she developed recurrent fever, arthralgia, rash and anasarca. She was suspected as having SLE with active lupus nephritis. Antinuclear antibody (ANA), and anti double stranded deoxyribonucleic acid (anti dsDNA) titers were positive and complements were low. Renal biopsy showed diffuse proliferative glomerulonephritis grade IV. She was treated with steroids and immunosuppressants to which she responded. Dengue viremia incites antibody production, which if excessive causes deposition of viral antigen-antibody immune complexes. This could possibly lead to renal tubular damage and glomerulonephritis in susceptible individuals. Dengue fever leading to development of glomerulonephritis is rarely seen. Our patient developed dengue fever and after a month presented with manifestations of SLE and lupus nephritis. Both dengue fever and SLE have common manifestations of fever, arthralgia, rash, leucopenia with thrombocytopenia and serositis. Bacterial and viral infections may act as a 'trigger' for starting or relapsing lupus activity in genetically predetermined individuals. In our case it may be possible that dengue virus could have triggered a dysfunctional immune response, resulting in the developing of autoimmunity and SLE with lupus nephritis.     

FcgammaRIIa/IIIa polymorphism and its association with clinical manifestations in Korean lupus patients

The aim of this study was to determine the distribution of the FcgammaRlla and FcgammaRIIIa polymorphisms and their association with clinical manifestations in Korean lupus patients. Three hundred SLE (systemic lupus erythematosus) patients (48 male, 252 female) meeting 1982 ACR criteria and 197 Korean disease-free controls were enrolled. Genotyping for FcgammaRlla 131 R/H and FcgammaRIIIa 176 F/V was performed by PCR of genomic DNA using allele-specific primers and the FcgammaRIIIa genotype was confirmed by direct sequencing of PCR product in some cases. There was significant skewing in the distribution of the three FcgammaRIIa genotypes between the SLE and the controls (P=0.002 for R/R131 vs R/H131 and H/H131, OR 2.5 (95% Cl 1.4-4.5), but not in FcgammaRIIIa genotypes. FcgammaRIIa-R allele was a significant predictor of lupus nephritis, as compared with SLE patients without nephritis (P=0.034 for R131 vs H131, OR 1.4 (95% Cl 1.03-1.9)), but proliferative nephritis (WHO class III and IV) was less common in patients with FcgammaRlla-R/R131 and in FcgammaRIIa-R allele. In 300 SLE patients, high binding allele combination H131/V176 was less common in SLE with nephritis than in SLE without nephritis. Hemolytic anemia was less common in R131/F176 allele combination among four FcgammaRIIa/FcgammaRIIIa allelic combinations. Male SLE patients showed a higher frequency of renal involvement, serositis, thrombocytopenia, malar rash and discoid rash than female SLE, and male SLE had a higher frequency of FcgammaRIIa-R/R131 or R131-allele than male controls, but FcgammaRIIa or FcgammaRIIIa genotypes had no association with renal involvement in male SLE patients. FcgammaRIIa-H/H131 showed a higher frequency of hemolytic anemia and less pulmonary complications in male SLE. Female SLE patients showed higher frequency of any hematologic abnormality, lymphopenia, anticardiolipin antibody (+) and anti-Ro antibody (+) than male SLE, and had earlier onset of first symptoms. There was no skewing in FcgammaRIIa or FcgammaRIIIa genotypes between female SLE and female controls, but FcgammaRIIa-R131 allele showed skewing between female SLE with nephritis and female SLE without nephritis. The age at onset of thrombocytopenia was earlier in FcgammaRIIa R/R131 among three FcgammaRIIa genotypes, and serositis in FcgammaRIIIa-F/F176 among three FcgammaRIIIa genotypes. FcgammaRIIa-R131 homozygote was a major predisposing factor to the development of SLE and FcgammaRIIa-RI31 homozygote and R131 allele were a predisposing factor, and H131/V176 was a protective allele combination in lupus nephritis. In contrast to other ethnic patients, in our study cohort, clinical manifestation was different between male and female, and FcgammaRIIa and FcgammaRIIIa showed somewhat different clinical associations between the genders.     

系统性红斑狼疮合并Evans综合征临床特点

目的总结系统性红斑狼疮(systemic lupus erythematosus,SLE)合并Evans综合征患者的临床特点。方法回顾性分析北京协和医院2004年1月至2012年7月SLE合并Evans患者的临床表现及实验室特点及治疗和预后。结果 SLE并发Evans综合征患者22例,占同期SLE住院患者3400例的0.65%。其中男3例,女19例,平均35.1岁(16~53岁)。22例患者中以血液系统受累[特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)或自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)]为首发表现的11例(50%),确诊SLE后诊断Evans综合征者6例,二者同时诊断的5例。SLE并发Evans综合征时,患者往往有多系统受累,表现为肾脏受累13例(59.1%),皮肤黏膜受累、关节炎各9例(40.9%),神经系统受累4例(18.2%),胃肠道、肺部受累各2例等。Evans综合征多发生于SLE活动期,患者平均狼疮活动指数评分(11.45±7.6)分(3~30分)。伴发其他结缔组织病5例(22.7%)。经激素联合免疫抑制剂治疗后,20例好转,2例无效者应用利妥昔单抗后好转。结论 SLE合并Evans综合征罕见,发生于SLE多系统受累及活动期。部分患者以ITP或AIHA为SLE首发表现,应及时筛查多种自身抗体,并定期随访密切观察,以期早期诊治。     

Systemic lupus erythematosus in Egyptian children

The aim of the study was to study the characteristics of systemic lupus erythematosus (SLE) in the Egyptian population, comparing it to other populations. We retrospectively studied 207 patients with SLE diagnosed between 1990 and 2005. We obtained clinical features and laboratory data and analyzed them statistically. We studied 151 female and 56 male SLE patients. The female to male ratio was 2.7 to 1 and the mean age at presentation was 10 ± 2.7 years (range 2–16). The mean disease duration was 6.47 ± 3.74 years. At diagnosis, musculoskeletal, constitutional and mucocutaneous manifestations were the commonest features. During follow-up, the prevalence of nephritis (67%), hematological manifestations (44.9%), photosensitivity (44%), arthritis (39%), malar rash (38.2%), serositis (32.9%) and neuropsychiatric manifestations (24.25%) increased significantly. Those whose age of onset of the disease was ≤5 years (nine patients) had significantly more common hematological affection (P value = 0.0005). The characteristics of SLE in Egyptian patients show some similarities to other series of Middle Eastern countries, but with a lower female to male ratio. Disease onset below 5 years is extremely rare (4.35%), commonly presenting with hematological manifestations. The kidney was the commonest major internal organ involved, and also an important cause of death.     

男性狼疮性肾炎的临床研究

目的探讨男性狼疮性肾炎的临床特点并与女性狼疮性肾炎患者进行比较。方法收集2004年1月—2008年9月住院的31例男性狼疮性肾炎患者和同期263例女性患者的临床资料进行统计学对比分析。结果（1）在肾脏受损方面：男性患者肾损害发生早且有较严重的肾损害;（2）肾外临床表现：男性关节疼痛、发热、多浆膜腔积液多于女性,而女性则以面部红斑、光过敏、雷诺现象、脱发为常见;（3）在实验室检查方面：男性有较严重的贫血、血小板减少而自身抗体阳性率、阳性滴度较低。结论男性与女性狼疮性肾炎的临床表现有较明显差别,临床上要注意男性狼疮性肾炎的早期诊断,及时治疗。     

Pediatric lupus in Asia

Of all patients with systemic lupus erythematosus (SLE), 15-20% are diagnosed during childhood, with disease onset prior to the age of 16 years. Because disease expression in SLE is influenced by environment factors and differs between racial and ethnic groups. The aims of this review were to describe prevalence, clinical manifestations, common infectious complications, and outcome of pediatric-onset SLE in Asia. The prevalence of pediatric-onset SLE was 6.3-19.3 per 100,000 in Asia. The ratio of female to male was 4.7-6.2. The mean age at diagnosis of pediatric-onset SLE was 8.6-13.5 years. The most common clinical features of pediatric-onset SLE in Asia were cutaneous rashes, arthritis, hematological involvement and nephritis. The occurrence of nephritis varies from 29% to 81%. The most common histopathology of lupus nephritis was diffuse proliferative glomerulonephritis (WHO Class-IV) which occurred in 39.4-54% of case of lupus nephritis. Pediatric-onset SLE patients with infections have poor outcomes than uninfected patients. Gram-negative bacilli are the most common microorganisms responsible for bacteremia in Asian patients with SLE. Recurrent major infections predict poorer disease outcome and associated organ damage in pediatric-onset SLE. Improving the survival of SLE patients was reported in Asia in recent decades. The survival was 92% at the age of 5 years, 86% at 10 years and 79% at 15 years in children with SLE in Taiwan in 2008.     

Clinical and laboratory variables of childhood systemic lupus erythematosus in western province of Saudi Arabia

To study the demographic, clinical, laboratory features, treatment and outcome of childhood systemic lupus erythematosus (SLE) in western province of Saudi Arabia. Children with SLE who were diagnosed at King Abdulaziz University Hospital, Jeddah, between March 1998 and October 2008 were included. Charts of all patients were reviewed retrospectively for clinical and laboratory features, treatment and outcome. There were 28 girls and 2 boys, with a mean age of 10.5 years (range 5–18). The female:male ratio was 14:1. Constitutional symptoms represent significant symptoms. Hematological manifestations were the most frequent finding (86.7%) at the time of diagnosis followed by arthritis and nephritis (73.3%). The malar rash represents the most common skin manifestation (46.7%). Discoid lupus was very rare. Neurological symptoms were seen in 30%, while cardiac and pulmonary involvement was uncommon. All patients had positive ANA and 90% of them had high anti-ds DNA. All patients were treated with steroids and hydroxychloroquine and 26 patients received immunosuppressive therapy. Three patients died due to severe infection; massive brain infraction and severe lung disease (one in each). Twenty-seven patients are alive in stable condition. Clinical manifestations and laboratory abnormalities were similar to previously reported series. This report confirms that SLE has comparable findings among children from different ethnicities.     

Systemic lupus erythematosus. III. Observations on clinical renal involvement and follow up of renal function: Dutch experience with 110 patients studied prospectively.

A prospective study of 110 patients with systemic lupus erythematosus (SLE) was undertaken to evaluate the reliability of clinical signs of lupus nephritis, which developed in 39 (35%) patients. Those patients with SLE who showed no clinical signs of lupus nephritis had an excellent survival rate (10 year survival 93%) and retained normal renal function (serum creatinine less than 130 mumols/l); clinical lupus nephritis developed mainly in the first three years after diagnosis of SLE and was associated with a decreased survival rate (10 year survival 62%). Increased mortality was found in male patients with lupus nephritis over 25 years of age and in female patients with lupus nephritis under 25 years of age, while renal failure rates did not differ between these groups. Treatment of lupus nephritis with high dose prednisone alone or in combination with immunosuppressants did not result in differences in patient survival or renal function preservation. It was concluded that clinical variables are a reliable guide in the management of patients with SLE, and routine use of renal biopsy in these patients is rejected.     

Clinical and immunological manifestations in 151 SLE patients living in Dubai

To gain better understanding of systemic lupus erythematosus (SLE) in Dubai we studied the clinical and immunological manifestations in a cohort of 151 patients attended Rheumatology Clinic in Dubai Hospital between January 2002 and January 2007. We found that the female to male ratio was 20.5:1, with a mean age of 35.5 years (0.9). The mean age at disease onset was 28.9 years (0.8) and mean disease duration 6.7 years (0.4). Five-year survival rate in our cohort was 94%. The commonest clinical manifestations in this cohort were arthritis (88%), haematological abnormalities (61.6%), and malar rash (60.3%). Leucopenia, fever, hair loss and proteinuria were observed in approximately half of the patients. Anaemia was found in 44.3% but only 9.9% had haemolytic anaemia. Photosensitive rash was seen in 43% of patients. Approximately one-third of the patients had serositis and mouth ulcers, 30.5 and 27.2% respectively. Vasculitis was observed in 19.2% of patients. Neuropsychiatric manifestations (15.9%), discoid lupus lesions (12.6%), and brain infarcts (13.2%) were infrequent. Subacute cutaneous lupus (6%) was also uncommon. Anti-nuclear antibodies were detected in 98%, anti-double stranded DNA antibodies in 88.7%, anti-Sm antibodies in 19.7%, anti-RNP in 40.4%, anti-Ro antibodies in 52.3% and anti-La antibodies in 19.8%. Anti-cardiolipin IgM and IgG were detected in 25.3 and 22.4%, respectively. This study suggests that Arabs with SLE residing in Dubai have comparable clinical features to their counterparts in other Arab countries and Western countries. The high prevalence of positive anti-Ro antibodies among our Arab patients probably reflects a character, that is, commonly seen in SLE patients of Middle East origin.