Clinical features of systemic lupus erythematosus

We compared the frequency of clinical features of systemic lupus erythematosus (SLE) and determined survival in 113 patients with younger-onset lupus (age ⩾ < at clinical diagnosis) and 25 patients with older-onset disease (age ⩾55 at diagnosis). The most striking difference was in the racial distribution; 59% of the younger patients were black, compared with only 20% of the older-onset patients (P < 0.001). Major manifestations of lupus (including clinically evident renal disease, central nervous system involvement, cutaneous involvement, and hemocytopenia) occurred with similar frequency in both age groups. Antibodies to DNA were detectable equally often in both groups, but hypocomplementemia was more common in the younger patients. Five-year survival in the younger-onset group (79%) was similar to that of the older-onset group (72%); there was a tendency toward relatively improved survival in patients in the latter group when compared with the expected survival of appropriately matched control populations. Major significant differences in racial distribution included 1) a higher incidence of serositis in older whites and in blacks regardless of age, and 2) more frequent hypocomplementemia in younger patients within both racial groups.     

Clinical features of chorea associated with systemic lupus erythematosus.

Neurologic complications of systemic lupus erythematosus (SLE) are common, but chorea is rare. Three cases of chorea associated with SLE are presented as well as a review of 28 cases from the world literature. Chorea may be the first and at times the only sign prior to the establishment of diagnosis of SLE. Under such conditions the establishment of the correct diagnosis is difficult, and the chorea may be attributed to a more common etiology, Also the SLE-related chorea may recur in the same subject during the course of the disease. Finally, the chorea associated with SLE can be brought under control by use of haloperidol (HALDAL).     

Clinical features of Pneumocystis pneumonia in patients with systemic lupus erythematosus]

Systemic lupus erythematosus (SLE) is often associated with various opportunistic infections, particularly during treatment with corticosteroids or immunosuppressants. We studied the clinical characteristics of 15 patients with SLE who underwent diagnostic bronchoalveolar lavage (BAL) and compared 6 patients with confirmed Pneumocystis pneumonia (PcP+), with 9 patients without Pneumocystis pneumonia (PcP-). The serum concentrations of beta-D-glucan and KL-6 were significantly higher in PcP+ than in PcP- patients, whereas serum LDH was similar in both groups. The serum concentrations of complement, a marker of SLE activity, and of IgG did not predict the presence of PcP. In all patients, the overall cell and lymphocyte counts were increased in the BAL fluid, without any significant difference between the PcP+ and PcP- groups. Ground-glass opacities on chest computed tomography, and oxygenation impairment (PaO2/FiO2<200Torr) were more common in PcP+ than PcP- patients. We concluded that, in patients with SLE, serum beta-D-glucan and KL-6 might be useful in the diagnosis of PcP, particularly when severe hypoxemia precludes BAL.     

Clinical features of interstitial pneumonia associated with systemic lupus erythematosus

BackgroundSystemic lupus erythematosus (SLE) commonly affects the lungs. However, the incidence of interstitial pneumonia (IP) related to SLE was reported to be about 10%, less than in the case of other connective tissue diseases, and the mechanism via which IP is related to SLE remains to be elucidated.MethodsWe retrospectively reviewed the medical records and high-resolution computed tomography (HRCT) images of 69 SLE patients who were admitted to our hospital between January 2011 and December 2015.ResultsFifty-five of the patients were female (80%), and the mean age at the onset of SLE was 42.4 years. IP developed in 20 patients (29%), 14 of whom were female (70%), and the mean age at SLE onset was 53.4 years, significantly older than those without IP (38.0 years) (p = 0.003). Half of the patients were found to have IP during the initial diagnosis of SLE. The IP pattern on the HRCT images was consistent with that of usual interstitial pneumonia (UIP) in 25% of the patients and of nonspecific interstitial pneumonia (NSIP) in 55%. One patient exhibited acute exacerbation but survived. The radiological findings revealed that the disease progressed slowly in most of the patients; however, pulmonary function was retained. No significant differences were observed in the survival rates between patients with and without IP.ConclusionIn SLE cases, IP primarily occurred in male and elderly patients. In addition to the NSIP pattern, the UIP pattern was evident on HRCT scans of IP-related SLE. The survival of SLE patients was unrelated to IP.     

Clinical features of reversible posterior leukoencephalopathy syndrome in patients with systemic lupus erythematosus

Abstract

To characterize reversible posterior leukoencephalopathy syndrome (RPLS) in systemic lupus erythematosus (SLE) in terms of treatments for resolution and its clinical course, we reviewed 28 cases of RPLS in SLE including our cases in view of the treatment. Of these, 15 cases improved with blood pressure control and 13 required immunosuppressive therapy for activity of SLE presenting neurological manifestations. Patients without immunosuppressants at onset of RPLS more frequently required immunosuppressive therapy to recover it than those precedingly using these agents [31% (4/13) versus 87% (13/15), p = 0.008, chi-square test]. Brain magnetic resonance imaging (MRI) is important for diagnosis of RPLS-SLE in the patient with SLE who develops neurological disturbance and rapidly increasing blood pressure. When 7-day therapy for hypertension and convulsion does not reverse the manifestations, immunosuppressive treatments would be recommended to reverse RPLS.     

系统性红斑狼疮合并乙型肝炎病毒感染临床分析

目的 了解系统性红斑狼疮(SLE)合并乙型肝炎病毒(HBV)感染患者临床特点.方法 回顾性分析2003年2月至2011年6月在北京协和医院住院的40例合并HBV感染的SLE患者临床资料,并与性别、发病年龄相匹配的60例无HBV感染的SLE患者比较.统计学方法采用t检验和x2检验.结果 40例合并HBV感染者中男性4例,女性36例,平均年龄(32±13)岁;其中23例患者表现为慢性乙型肝炎,5例为HBV携带状态,12例为既往无症状感染.21例慢性乙型肝炎患者肝功能轻度异常主要为丙氨酸转氨酶升高,2例严重肝功能异常.临床表现合并HBV感染组和对照组比较,发热、肝脏受累、肾脏受累、血小板减少、巨细胞病毒感染更常见.肾病变主要为Ⅳ型或Ⅴ型狼疮肾炎,2例合并HBV相关性肾炎.血清乙型肝炎病毒表面抗原(HBsAg)阳性的SLE患者在接受免疫抑制剂治疗时同时使用拉米夫定抗病毒治疗.患者出院好转率为98％ (39/40),1例预后不佳者有严重肝脏病变.结论 应重视SLE合并HBV感染患者临床特点:SLE合并HBV感染患者发热、肝脏受累、肾脏受累、血小板减少、巨细胞病毒感染者增多,可合并HBV相关性肾炎;肝脏病变严重者预后差;血清HBsAg阳性的SLE患者在接受免疫抑制剂治疗时应同时使用抗HBV药治疗.     

Clinical features of reversible posterior leukoencephalopathy syndrome in patients with systemic lupus erythematosus

To characterize reversible posterior leukoencephalopathy syndrome (RPLS) in systemic lupus erythematosus (SLE) in terms of treatments for resolution and its clinical course, we reviewed 28 cases of RPLS in SLE including our cases in view of the treatment. Of these, 15 cases improved with blood pressure control and 13 required immunosuppressive therapy for activity of SLE presenting neurological manifestations. Patients without immunosuppressants at onset of RPLS more frequently required immunosuppressive therapy to recover it than those precedingly using these agents [31% (4/13) versus 87% (13/15), p = 0.008, chi-square test]. Brain magnetic resonance imaging (MRI) is important for diagnosis of RPLS-SLE in the patient with SLE who develops neurological disturbance and rapidly increasing blood pressure. When 7-day therapy for hypertension and convulsion does not reverse the manifestations, immunosuppressive treatments would be recommended to reverse RPLS.     

系统性红斑狼疮患者结核菌感染的临床特点及相关因素分析

目的分析系统性红斑狼疮（SLE）患者结核菌感染的临床表现、特点以及相关危险因素。方法回顾分析了37例SLE结核感染住院患者和65例随机抽取的SLE对照组患者的临床资料．所有患者均有完整的病史、体格检查、实验室等辅助检查的资料。结果在37例SLE结核感染患者中．肺结核13例（35％），肺结核合并其他部位结核者7例，肺外结核17例。与对照组相比，结核感染组的日平均激素的剂量和细胞毒药物使用较高，存两组之间差异有统计学意义，而性别、年龄、病程、白蛋白／球蛋白、补体和激素冲击治疗等方面，两组问差异无统计学意义。讨论SLE患者发生肺外结核感染的机会较大，且日平均激素的剂量和细胞毒药物是SLE患者发生结核感染的危险因素。     

Clinical and radiological features of osteonecrosis in systemic lupus erythematosus.

Symptomatic osteonecrosis occurred in 8 out of 68 patients with systemic lupus erythematosus. Multiple joints were involved in 3 patients, and weight-bearing joints were most frequently affected. Osteonecrosis tended to occur early in the disease, and the patients had all received corticosteroids. Symptoms tended to occur when the disease had progressed from an active phase into one of clinical and serological quiescence. In weight-bearing joints classical radiological changes were often absent at the onset of symptoms.     

系统性红斑狼疮患者活动性发热特点及相关性分析

目的 探讨系统性红斑狼疮(SLE)患者活动性发热的临床特点并进行相关性分析.方法 由各参研单位根据统一方式收集病历资料,总结江苏省1999-2009年住院SLE患者的临床资料及治疗情况,分析发热与SLE的临床表现、免疫学指标、疾病活动及治疗情况间的关系.组间的计数资料比较采用x2检验,非正态分布的计量资料比较采用秩和检验,相关性分析采用Logistic回归分析.结果 1762例SLE患者纳入研究范围,其中活动性发热患者729例,非发热患者1033例,通过对照研究,发热组入院年龄、是否初治、光敏感、浆膜炎、神经精神系统累及、肝脾淋巴结肿大、血白细胞、血红蛋白、红细胞沉降率(ESR)、C反应蛋白(CRP)、丙氨酸转氨酶(ALT)、白蛋白、血清肌酐、补体C3、抗双链DNA (dsDNA)抗体、抗Sm抗体、SLE疾病活动指数(SLEDAI)评分、既往治疗与非发热组比较差异有统计学意义(尸＜0.05);Logistic回归分析显示血白细胞异常[比值比(OR)=1.396,95％可信区间(CI)1.114～1.711,P=0.004]、CRP (OR=1.005,95％CI 1.002～1.009,P=0.002)、ALT( OR=1.003,95％CI 1.001 ～1.005,P=0.005)、血清肌酐(OR=0.997,95％CI 0.995～0.999,P=0.007)、血红蛋白(OR=0.986,95％CI0.981～0.992,P=0.000)、入院年龄(OR=0.984,95％CI 0.974～0.993,P=0.001)及环磷酰胺应用比例(OR=0.557,95％CI 0.382～0.813,P=0.002)与SLE 患者发热相关.结论 发热是SLE常见的临床表现之一.血白细胞下降、CRP升高、肝功能异常、贫血、入院年龄轻为SLE患者出现发热症状的危险因素,而肾累及、环磷酰胺应用为保护因素.     

Damage in cuban patients with systemic lupus erythematosus. Relation with disease features

ObjectiveTo determine damage presence and predictors factors for its appearance in a cohort of cuban patients with systemic lupus erythematosus (SLE).Patients and methodsA retrospective cohort study included 80 patients presenting with SLE seen in Rheumatology Service of “Hermanos Ameijeiras” Clinical Surgical Hospital in Havana City, Cuba. Damage was assessed using The Systemic Lupus International Collaborating Clinics/American College of Rheumatology (ACR) Damage Index (SLIC/ACR), a tool approved for damage measurement. Damage presence was related to initial disease features to diagnose this condition, to sociodemographic elements, to treatments used, and to the disease course time. Statistical analysis had two variants: the univariate and multivariate type using Chi2 and statistical significance was established in p<0, 05.ResultsWe found that 39 patients (48,8%) had some degree of damage. More involved domains were the musculoskeletal (18,8%), neuropsychiatric, and skin, 16,3%, pulmonary and ocular, present in 15% of cases. In the multivariate analysis, damage was associated with the use of higher than 30 mg/day Prednisone doses for more of 4 weeks (OR=54,68, CI 95%=3,56–97,45, p=0.001), presence of leukopenia (RO=18,73, CI 95%=2,74–62,23 m p=0,004), and time course of disease (OR=1,02, CI 95%=1,00 2–1,09, p=0.006). Conclusions: Damage was practically present in half of the study patients, the most involved domain was the musculoskeletal, and use of higher than 30 mg prednisone doses were the factor most associated with the presence of damage.     

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.     

34例神经精神狼疮患者的临床资料分析

目的:对神经精神狼疮(NPSLE)患者的临床表现、治疗及预后进行总结。方法:对34例确诊为NPSLE住院患者的临床表现、影像学资料、实验室指标、治疗及预后等临床资料进行回顾性分析。结果:同期住院诊断SLE共818例,诊断NPSLE34例,发病率为4.16%。NPSLE首发症状最常见的是狼疮性头痛(24.2%)、脑血管病变(22.3%),发热(97.1%)及新发皮疹(61.8%)为常见的伴随症状。根据SLEDAI评分,34例患者中76.5%患者处于疾病重度活动期(评分≥15)。结论:NPSLE临床表现多样,2种及以上临床亚型并存者多见。首发症状以狼疮性头痛最常见。颅脑影像学检查在NPSLE的诊断中具有重要意义。     

Cutaneous lupus erythematosus without systemic lupus erythematosus: clinical and laboratory features.

Sixty-seven patients with cutaneous lupus erythematosus (CLE) were followed up as part of a series of 570 lupus erythematosus patients seen in a private practice between 1980 and 1989. Clinical and laboratory features, treatment, and natural course were observed. Findings of interest included (1) a ratio of at least one CLE case for every seven cases of systemic lupus erythematosus (SLE); (2) occurrence of CLE in fewer women and apparently associated with an older age at diagnosis than SLE; (3) similar frequency of cutaneous lupus subsets in CLE and SLE; (4) strong family history for SLE but not CLE in CLE patients; (5) other cutaneous and musculoskeletal features in a majority of CLE patients and constitutional symptoms in 10%; (6) positive ANA titers, high sedimentation rates, and leukopenia common in CLE; (7) anticardiolipin antibody in 31% of CLE patients but not associated with systemic complications; (8) antimalarials required by 75% of patients and systemic steroids by 33%; and (9) an excellent prognosis associated with CLE, organ-threatening disease being rare.     

小剂量利妥昔单抗治疗系统性红斑狼疮的临床观察

目的 评价小剂量利妥昔单抗治疗系统性红斑狼疮(SLE)的疗效和安全性.方法 对既往激素和多种免疫抑制剂治疗无效或复发的10例SLE患者,予利妥昔单抗100mg,使用4次,同时仅联合使用激素,观察疗效和不良反应.结果 利妥昔单抗对狼疮肾炎、神经精神性狼疮、顽固性血小板减少以及自身免疫性溶血性贫血均显示有良好的疗效,且起效迅速.10例患者中仅1例出现了泌尿系感染,1例在首剂利妥昔单抗治疗1周后猝死,但不能确定死亡原因与利妥昔单抗相关.结论 小剂量利妥昔单抗治疗SLE有良好的疗效和安全性,并且降低了治疗费用.     

Clinical significance of anticentromere antibodies in patients with systemic lupus erythematosus

OBJECTIVE: To clarify the clinical significance of anticentromere antibodies (ACA) in patients with systemic lupus erythematosus (SLE). METHODS: Two hundred sixteen patients with SLE who were treated in our department were surveyed cross sectionally for the presence of ACA using indirect immunofluorescence on HEp-2 cell lines. ACA were identified by their discrete speckled pattern. Antibodies to the major centromere protein, CENP-B, were also studied with ELISA. Serial determinations of anti-CENP-B were carried out using stored serum samples, if available. RESULTS: ACA were recognized in 12 (5.6%) patients with SLE. All patients were receiving steroid therapy, with a mean dose of prednisolone of 14.4 mg/day. These patients also tested positive for anti-CENP-B with high titers despite the low serological disease activity in most. Three or more CREST features were observed in 2 patients and 2 others had no such features. Both patients without CREST features had a relatively short disease duration. The age at onset of SLE was significantly higher and Raynaud's phenomenon was more frequent in patients with ACA than in patients without ACA. In 8 of 10 patients tested, retrospective analysis using stored sera revealed no consistent change in anti-CENP-B titers over time. CONCLUSION: The presence of ACA in patients with SLE is apparently more frequent than previously believed. Patients with SLE with ACA may be a distinct subgroup. A longterm followup is warranted to fully determine the clinical significance of ACA in patients with SLE.     

Clinical associations of antiribonucleoprotein antibodies in patients with systemic lupus erythematosus

The authors undertook a cross-sectional study to investigate the clinical associations of antiribonucleoprotein (anti-RNP) antibodies in 49 patients with systemic lupus erythematosus (SLE) without other concomitant connective tissue disorders. The traditional counterimmunoelectrophoresis (CIE) and the immunoblotting (IB) technique were compared. Clinically, special attention was given to the identification of sclerodermalike features. All patients completed a detailed questionnaire, physical examination, and additional investigations including pulmonary function tests, chest roentgenogram, radionuclide transit studies of the esophagus, and nailfold capillary microscopy. Pulmonary function testing and radionuclide transit studies of the esophagus were very sensitive for the detection of (subclinical) pulmonary and esophageal involvement, respectively. Within the relatively homogeneous SLE population, a subset was recognized that was characterized clinically by the presence of sclerodermalike features such as Raynaud's phenomenon, sclerodactyly, interstitial changes on chest roentgenogram, and decreased numbers of nailfold capillary loops, and serologically by the presence of anti-RNP antibodies. IB was somewhat more sensitive than CIE for the detection of anti-RNP (anti-Sm/anti-nRNP) antibodies but did not identify other clinical associations. Thus, anti-RNP antibodies in SLE are associated with scleroderma-associated features. For clinical practice, CIE is the technique recommended for their detection.