系统性红斑狼疮患者动脉脉搏波速度的临床研究

目的:研究系统性红斑狼疮(SLE)患者动脉脉搏波速度及其影响因素。方法:选择66例SLE患者(SLE组)和33例正常对照者(正常对照组)应用脉搏波速度(PWV)测量仪测定颈动脉—股动脉PWV(C-F PWV)及颈动脉—桡动脉PWV(C-R PWV)作为反应动脉扩张性的指标。SLE患者同时记录疾病活动情况及治疗相关参数。结果:C-F PWV和C-R PWV在SLE组显著升高,分别为(8．4±1．5)m/s,(9．8±1．9)m/s,正常对照组的C-F PWV和C-R PWV分别为(7．5±1．1)m／s,(8．0±1．0)m／s,P值分别为0．0007,0．0001,SLF组脉压明显高于正常对照组,分别为(43．0±8．5)mmHg,(38．4±6．2)mmHg,P值为0．0074。结论:本研究首次显示了SLE患者的PWV显著升高,提示SLE患者存在动脉扩张性的降低。     

Clinical and Serologic Features in Patients With Incomplete Lupus Classification Versus Systemic Lupus Erythematosus Patients and Controls

Objective

Incomplete lupus erythematosus (ILE) involves clinical and/or serologic manifestations consistent with but insufficient for systemic lupus erythematosus (SLE) classification. Because the nature of ILE is poorly understood and no treatment recommendations exist, we examined the clinical manifestations, medication history, and immunologic features in a diverse collection of ILE and SLE patients.

Methods

Medical records of subjects enrolled in the Lupus Family Registry and Repository were reviewed for medication history and American College of Rheumatology (ACR) classification criteria to identify ILE patients (3 ACR criteria; n = 440) and SLE patients (≥4 ACR criteria; n = 3,397). Participants completed the Connective Tissue Disease Screening Questionnaire. Anticardiolipin and plasma B lymphocyte stimulator (BLyS) were measured by enzyme‐linked immunosorbent assay, antinuclear antibodies (ANAs) by indirect immunofluorescence, and 13 autoantibodies by bead‐based assays.

Results

On average, ILE patients were older than SLE patients (46.2 years versus 42.0 years; P < 0.0001), and fewer ILE patients were African American (23.9% versus 32.2%; P < 0.001). ILE patients exhibited fewer autoantibody specificities than SLE patients (1.3 versus 2.6; P < 0.0001) and were less likely to have ANA titers ≥1:1,080 (10.5% versus 19.5%; P < 0.0001). BLyS levels were intermediate in ILE patients (controls < ILE; P = 0.016; ILE < SLE; P = 0.008). Pericarditis, renal, or neurologic manifestations occurred in 12.5% of ILE patients and were associated with non–European American race/ethnicity (P = 0.012). Hydroxychloroquine use increased over time, but was less frequent in ILE than SLE patients (65.2% versus 83.1%; P < 0.0001).

Conclusion

Although usually characterized by milder symptoms, ILE manifestations may require immunomodulatory treatments. Longitudinal studies are necessary to understand how ILE affects organ damage and future SLE risk, and to delineate molecular pathways unique to ILE.

     

系统性红斑狼疮合并主动脉夹层1例及文献复习

目的:探讨系统性红斑狼疮(SLE)合并主动脉夹层(AD)患者的临床特点,夹层形成的病理生理机制及本病的治疗.方法:分析我院收治的1例SLE合并AD以及1969年至今的国内外文献报道共29例.结果:29例中大多为中青年,长期服用激素治疗,大多数伴有高血压.按Stanford分型A型19例,手术治疗8例,全部存活;保守治疗11例,均死亡.B型10例,手术治疗5例(包括1例介入治疗),存活3例;保守治疗5例,存活1例.结论:长期激素治疗、SLE相关的高血压、动脉粥样硬化共同促进了AD的发生.手术治疗的存活率明显高于保守治疗者.     

Brief Report: Attention Deficit and Hyperactivity Disorder Scores Are Elevated and Respond to N‐Acetylcysteine Treatment in Patients With Systemic Lupus Erythematosus

Objective

To investigate whether attention deficit hyperactivity disorder (ADHD) may serve as a marker of neuropsychiatric disease and as a target for N‐acetylcysteine (NAC) treatment in patients with systemic lupus erythematosus (SLE).

Methods

The ADHD Self‐Report Scale (ASRS) was used to assess 49 patients with SLE and 46 matched healthy control subjects. Twenty‐four of the patients with SLE were randomized to receive either placebo, NAC at a dosage of 2.4 gm/day, or NAC at a dosage of 4.8 gm/day. Disease activity was evaluated monthly using the British Isles Lupus Assessment Group (BILAG) index, the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), the Fatigue Assessment Scale (FAS), and the ASRS, before and during the 3‐month treatment period and after a 1‐month washout period.

Results

The cognitive/inattentive (ASRS part A), hyperactivity/impulsive (ASRS part B), and combined (total) ASRS scores were increased in patients with SLE compared with control subjects (mean ± SEM 17.37 ± 1.03 [P = 3 × 10⁻⁷], 14.51 ± 0.89 [P = 2 × 10⁻⁴], and 31.92 ± 1.74 [P = 8 × 10⁻⁷], respectively, versus 10.41 ± 1.02, 9.61 ± 1.21, and 20.02 ± 1.98, respectively. ASRS part A scores correlated with SLEDAI (r = 0.53, P < 0.0001) and BILAG scores (r = 0.36, P = 0.011). ASRS total scores also correlated with SLEDAI (r = 0.45, P = 0.0009) and BILAG scores (r = 0.31, P = 0.025). ASRS part A (r = 0.73, P < 0.0001), ASRS part B (r = 0.47, P = 0.0006), and ASRS total scores (r = 0.67, P < 0.0001) correlated with the FAS score. Relative to the scores in placebo‐treated patients, ASRS total scores were reduced in SLE patients treated with NAC dosages of 2.4 gm/day and 4.8 gm/day combined (P = 0.037). ASRS part A scores were reduced by NAC dosages of 2.4 gm/day (P = 0.001) and 4.8 gm/day (P < 0.0001) as well as by NAC at dosages of 2.4 gm/day and 4.8 gm/day combined (P = 0.001).

Conclusion

In patients with SLE, elevated ASRS scores reveal previously unrecognized and clinically significant symptoms of ADHD that respond to NAC treatment.

     

Incidence of Cancer in a Cohort of Spanish Patients With Systemic Lupus Erythematosus

ObjectiveTo determine the incidence and prevalence of cancer in a cohort of patients with systemic lupus erythematosus (SLE) and identify associated risk factors.Patients and methodsThe study comprised a dynamic cohort of SLE patients (November 1989 to December 2006) at a tertiary referral centre. An adjusted external control from the hospital tumour registry was used.ResultsThe study included 175 SLE patients (90% women), with a mean time at risk of 1370.5 patient-years. Fourteen women (8%) died, mainly from cardiovascular events. No patient died due to malignancy. We found 35 tumours in 28 patients, 25 of them after the diagnosis of SLE, of which 5 were malignant. The rate of benign tumours was 14.6/1000 patient-years (95% CI, 8.9–22.5) and of malignant tumours 3.6/1000 patient-years (95% CI, 1.5–8.8), with a crude incidence odds ratio for malignant tumours of 3.5 (95% CI, 1.5–7.9). However, this significance was lost after standardizing the rates. Concerning associated risk factors, differences were found in the mean erythrocyte sedimentation rate [HR 1.4 (1.1–1.7)], and the presence of thrombosis [HR 6.9 (1.49–41.2)], especially arterial thrombosis.ConclusionsWe found a crude incidence rate of cancer that was almost four times greater in our SLE patients as compared with the expected rate in the hospital area of western Malaga.     

Serologic Studies In Patients With Systemic Lupus Erythematosus And Central Nervous System Dysfunction

Serologic studies were performed on 25 patients with systemic lupus erythematosus (SLE) during 29 acute episodes of central nervous system (CNS) disease. Increased anti-DNA antibody and decreased total serum hemolytic complement activity were observed only in those patients with associated extra-CNS disease manifestations. Patients with isolated CNS disease were otherwise in apparent clinical and serological remission regarding these two indices. No special association of cold-reactive IgM antilymphocyte antibodies was demonstrable in patients with ongoing CNS injury. Of special interest was an increased incidence of anti-Sm antibodies in the patients with CNS dysfunction relative to that in a large group of patients without neuropsychiatric disease. The incidence of anti-RNP was not increased. The data do not support direct involvement in SLE brain injury of either DNA/anti-DNA complexes or of lymphocytotoxic antibodies cross-reactive with brain cells, but do suggest an association of anti-Sm with CNS disease in this disorder.