The reliability of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index in patients with Systemic Lupus Erythematosus

Objective. To test the reliability of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index and the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) in the assessment of patients with SLE. Methods. Ten patients with SLE, representing a spectrum of damage and activity, were included. Each patient was examined by 6 of 10 physicians from 5 countries, representing 10 lupus clinics. The SLICC/ACR Damage Index was used to assess accumulated damage, and the SLEDAI was used to assess disease activity. The order of the patients and physicians was randomized according to a Youden square design. Results. The SLICC/ACR Damage Index detected differences among patients (P < 0.001). There was no detectable observer difference (P = 0.933), and there was no order effect (P = 0.261). Similar results were obtained with the SLEDAI. There was concordance in the SLICC/ACR Damage Index among observers, despite a wide spectrum of disease activity detected by the SLEDAI. Conclusion. Physicians from different centers are able to assess patients with SLE in a reproducible way, using the SLEDAI to assess disease activity and the SLICC/ACR Damage Index to assess accumulated damage.     

Toward electronic health recording: evaluation of electronic patient reported outcome measures (e-PROMs) system for remote monitoring of early systemic lupus patients

The study aimed to assess the value of evaluation of electronic patient reported outcome measures (e-PROMs) in the assessment and management of SLE disease activity flares, its association with adherence to therapy as well as organ damage. A randomized, controlled crossover study was carried out over a 24-month duration. One hundred forty-seven SLE patients meeting the revised American College of Rheumatology (ACR) criteria were enrolled. Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was used to assess disease activity, whereas organ damage was scored using the Systemic Lupus International Collaborating Clinics (SLICC)/ACR Damage Index. In the first 12 months, the patients were assessed every 3 months. At 12 months, the patients were randomized into a cohort of 73 patients who continued their care in the same style and 74 patients who completed an online e-PROMs questionnaire on monthly basis for another 12-month period. The data captured were then retrospectively analyzed at the end of the 24-month study period. At the end of the first year of the study, the mean SLEDAI and SDI scores were 8.72 (6.1) and 1.9 (2.2). At the end of the second year, the mean SLEDAI and SDI scores in the e-PROMs cohort were 3.1 (2.6) and 1.2 (1.3), whereas in the control group, the scores were 7.63 (6.7) and 1.8 (2.3), respectively (p < 0.01). Adjusting for possible confounding variables, the number of flares, regardless of their severity, was associated with damage accrual (OR 2.03, 95% CI 1.34 to 2.83, p < 0.001). Adherence to therapy was significantly (p < 0.1) higher in the e-PROMs group. e-PROMs was equivalent to PROMs paper format and has a potential disease-modifying effect as it facilitated close monitoring of disease activity with an option of management escalation whenever indicated.     

Outcome of a national Israeli cohort of pediatric systemic lupus erythematosus

The aim of this study was to describe the clinical manifestations and outcomes of a national cohort of childhood systemic lupus erythematosus (cSLE). All cases of cSLE registered in the Israeli national registry of children with rheumatic diseases between 1987-2003 were examined for disease activity and damage by the SLE disease activity index (SLEDAI) and SLE collaborating clinics/American College of Rheumatology (SLICC/ACR) damage index. Demographic, clinical, laboratory and treatment factors were analysed for their effect on the outcome. One-hundred and two patients were identified, 81% females, with a mean age at diagnosis of 13.3 +/- 2.6 years. The mean SLEDAI score was 17.2 +/- 9.0 (range 2-60). Fifty four patients were followed for at least five years. The mean SLEDAI decreased to 7.6 +/- 6.3 (0-29) and the mean SLICC/ACR damage index was 0.7 +/- 1.6 (0-8). Five patients developed chronic renal failure. No patients died. No factors were found to be significantly associated with the outcome except the initial SLEDAI score. The five-year outcome of our national cSLE cohort was good; with relatively low activity and minimal damage in most patients. The initial SLEDAI predicted the development of late damage.     

Cigarette smoking and disease activity in systemic lupus erythematosus

OBJECTIVE: To investigate the effect of cigarette smoking on disease activity and cumulative organ damage in systemic lupus erythematosus (SLE). Methods. Extensive clinical and demographic variables, including current and previous cigarette smoking, were collected from 111 SLE patients using a detailed interview-administered questionnaire. Disease activity was estimated with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Cumulative organ damage was measured by the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR). Smoking status was correlated with disease activity and cumulative organ damage, while statistically adjusting for the individual effects of potentially confounding demographic and clinical variables using analysis of variance followed by Fisher's least significant difference method. Results. Current smokers demonstrated significantly higher (p < 0.001) SLEDAI scores (15.6 +/- 7.8) than ex-smokers (9.63 +/- 6.00), and never smokers (9.03 +/- 5.75). This association remained significant (p = 0.001) after adjusting for all covariates, including ethnicity, education level, income level, alcohol use, age of onset of SLE, current age, mean duration of SLE, marital status, and hydroxychloroquine therapy. Current smokers also demonstrated significantly (p = 0.003) higher scores for both the neurological and non-neurological components of SLEDAI. There was no significant difference in the SLICC/ACR scores across the various smoking groups, although there was a trend for more severe disease in current smokers. Conclusion. Cigarette smoking is associated with increased disease activity in SLE. These data further establish the association of SLE with cigarette smoking, and suggest that individuals with SLE should avoid all exposure to tobacco products.     

Smoking,disease activity,permanent damage and dsDNA autoantibody production in patients with systemic lupus erythematosus

The aim was to study the association of smoking with the activity and severity of systemic lupus erythematosus (SLE) and the production of antibodies to dsDNA. The study included 223 SLE patients attending the outpatient clinics at Helsinki University Central Hospital. The history of smoking was obtained by personal interview, and clinical data related to SLE by interview, clinical examination and chart review. The activity of SLE was assessed by the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score and permanent damage by the SLICC/ACR score. Antibodies to dsDNA were determined by three ELISA assays, by the indirect immunofluorescence technique using Crithidia luciliae cells as substrates and by the Farr assay. There were no significant differences in the SLEDAI scores between current smokers (73 patients), ex-smokers (59) and never-smokers (91), though current smokers tended to have lower disease activity. The SLICC/ACR scores between the groups were practically equal. Current smokers had significantly lower levels of antibodies to dsDNA than ex- and never-smokers (p = 0.025). Our study suggests that cigarette smoke may have immunosuppressive effect on autoantibody production in patients with SLE. Permanent damage was not found to be associated with smoking.     

Risk factors for damage in childhood-onset systemic lupus erythematosus: cumulative disease activity and medication use predict disease damage

OBJECTIVE: The Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index measures damage in adult patients with systemic lupus erythematosus (SLE), but its usefulness in patients with childhood-onset SLE has not been examined. This study was conducted to evaluate the sensibility of the SLICC/ACR Damage Index, to investigate how cumulative disease activity is related to damage in childhood-onset SLE, and to identify other risk factors for damage in childhood-onset SLE. METHODS: Disease activity and damage in 66 patients with newly diagnosed childhood-onset SLE were assessed retrospectively, and information on potential risk factors for damage (age, race, sex, medications, duration of disease, hypertension, body mass index, antiphospholipid antibodies, kidney disease, acute thrombocytopenia) was obtained. In addition, a group of physicians was surveyed to establish the sensibility of the SLICC/ACR Damage Index in childhood-onset SLE. RESULTS: The SLICC/ACR Damage Index was found to have face, content, and construct validity when used in children. The mean SLICC/ACR Damage Index score of the patients was 1.76 (mean followup 3.3 years). Cumulative disease activity over time was the single best predictor of damage (R(2) = 0.30). Other, possibly important risk factors for damage were corticosteroid treatment, the presence of antiphospholipid antibodies, and acute thrombocytopenia. It was determined that immunosuppressive agents may be protective. CONCLUSION: The SLICC/ACR Damage Index, though useful in childhood-onset SLE, may benefit from the introduction of weightings and redefinition of some of the items. Ongoing disease activity leads to disease damage, and treatment should be prompt. Prolonged use of high-dose corticosteroids may further increase damage, but use of immunosuppressive agents may protect against disease damage; this latter finding may have potential implications for the treatment of childhood-onset SLE and deserves further study. The relationship between disease activity and concomitant use of medication also requires further investigation.     

系统性红斑狼疮患者骨矿物质密度的研究

目的：了解系统性红斑狼疮（SLE)患者骨矿物质密度（BMD）的变化，并讨论病的病情和激素对骨密度的影响。方法：测定健康对照组、初诊SLE组（A组）、SLE激素治疗组（B组）的BMD、血钙、磷、骨特异性碱性磷酸酶（ALP）和甲状旁腺激素（PTH）。并将B组病人分为骨质疏松组和非骨质疏松组，对两组的病程、体重指数、发病年龄、疾病的活动指数（SLEDAI）、系统损害的指数（SLICC／ACR DI）、激素的累积剂量和当前量进行统计学分析。结果；A组与正常对照组比较各项 指标差异无显著性（P＞0．05）；B组腰椎BMD、骨特异性ALP均显著低于正常对照组（P＜0．05），PTH显著高于正常对照组（P＜0．05）。B组骨质疏松的发生率17．4％。骨质疏松组与非骨质疏松组比较有较长的病程（P＜0．01）、较高的累积激素用量（P＜0．01）和较高的SLICC／ACR DI（P＜0．05）。结论：骨质疏松是年轻的SLE患者的常见病变，疾病的病程越长和系统损伤指数越高伴随骨质疏松的危险越大，但激素的影响与骨质疏松的发生是有关的。     

Metabolic syndrome and insulin resistance comorbidity in systemic lupus erythematosus

Objective

The aim of the present study was to assess the effect of metabolic syndrome (MetS) and insulin resistance comorbidity on the carotid intima-media thickness (IMT) in systemic lupus erythematosus (SLE) patients and their relationship to clinical manifestations, disease activity, and damage.

Methods

The study included 92 SLE patients (mean age 30.18?±?8.27 years) and 30 matched controls. Disease activity and damage were assessed by the SLEDAI and SLICC indices, respectively. The Health Assessment Questionnaire II (HAQII) and Quality of Life (QoL) index were evaluated in the patients. Levels of insulin, glucose, and creatinine and the lipid profile were measured in patients and controls. Insulin sensitivity was estimated using the homeostatic model assessment index (HOMA-B) for beta cell function and (HOMA-IR) for peripheral tissue insulin resistance. The carotid IMT was measured by ultrasonography.

Results

The SLE patients had high HOMA-IR and HOMA-B. The IMT was significantly increased (0.82±?0.29 mm) compared to the controls (0.45±?0.2 mm).The HOMA-IR, SLEDAI, SLICC, HAQII, and IMT were significantly higher and the QoL lower in those with MetS (n?=?34) compared to those without (n?=?58), while the HOMAB was comparable. There was a significant correlation between the IMT and the SLEDAI, SLICC, and WHR.

Conclusion

Insulin sensitivity and IMT are altered in SLE patients, especially those with MetS comorbidity with an associated increase in disease activity and damage. Effective management of MetS would help control SLE activity, damage, and the future development of cardiovascular events especially in the absence of symptoms of cardiovascular disease.     

Urinary soluble VCAM-1 in systemic lupus erythematosus: a clinical marker for monitoring disease activity and damage

OBJECTIVE: To determine the urinary levels of soluble vascular cell adhesion molecule-1 (VCAM-1) and intercellular adhesion molecule-1 (ICAM-1) in patients with systemic lupus erythematosus (SLE) and to assess their relationship with clinical and laboratory features and the degree of activity and damage associated with the disease. METHODS: The study sample included 24 consecutive patients with SLE. 24-hour urine samples were collected for the determination of soluble VCAM-1 and ICAM-1 levels by ELISA. Disease activity was defined by the SLE Disease Active Index (SLEDAI) and disease outcome by the Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ ACR) damage index. RESULTS: The urinary soluble VCAM-1 level was significantly higher in patients with SLE compared to normal controls (32.35+/-34.27 vs. 4.66+/-3.8 ng/mg creatinine, p = 0.0005) and statistically significantly correlated with disease activity (SLEDAI), a low serum C3 level, decreased creatinine clearance and albuminuria, as well as with disease damage (SLICC/ACR damage index). In contrast, the urinary soluble ICAM-1 level was not significantly higher in the patients' group compared with the controls (4.5+/-5.19 vs. 2.72+/-2.31 ng/mg creatinine, p=0.2), but was statistically significantly correlated with hematuria and albuminuria. CONCLUSION: Our data suggest that the urinary level of soluble VCAM-1 significantly correlates with overall disease activity and damage scores, but not with nephritis in SLE.     

Serum uric acid levels contribute to new renal damage in systemic lupus erythematosus patients

This study aims to determine whether uric acid levels contribute to new renal damage in systemic lupus erythematosus (SLE) patients. This prospective study was conducted in consecutive patients seen since 2012. Patients had a baseline visit and follow-up visits every 6 months. Patients with ≥2 visits were included; those with end-stage renal disease (regardless of dialysis or transplantation) were excluded. Renal damage was ascertained using the SLICC/ACR damage index (SDI). Univariable and multivariable Cox-regression models were performed to determine the risk of new renal damage. Uric acid was included as a continuous and dichotomous (per receiving operating characteristic curve) variable. Multivariable models were adjusted for age at diagnosis, disease duration, socioeconomic status, SLEDAI, SDI, serum creatinine, baseline use of prednisone, antimalarials, and immunosuppressive drugs. One hundred and eighty-six patients were evaluated; their mean (SD) age at diagnosis was 36.8 (13.7) years; nearly all patients were mestizo. Disease duration was 7.7 (6.8) years. Follow-up time was 2.3 (1.1) years. The SLEDAI was 5.2 (4.3) and the SDI 0.8 (1.1). Uric acid levels were 4.5 (1.3) mg/dl. During follow-up, 16 (8.6%) patients developed at least one new point in the renal domain of the SDI. In multivariable analyses, uric acid levels (continuous and dichotomous) at baseline predicted the development of new renal damage (HR 3.21 (1.39–7.42), p 0.006; HR 18.28 (2.80–119.48), p 0.002; respectively). Higher uric acid levels contribute to the development of new renal damage in SLE patients independent of other well-known risk factors for such occurrence.     

Risk factors for damage in childhood‐onset systemic lupus erythematosus: Cumulative disease activity and medication use predict disease damage

Objective

The Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index measures damage in adult patients with systemic lupus erythematosus (SLE), but its usefulness in patients with childhood‐onset SLE has not been examined. This study was conducted to evaluate the sensibility of the SLICC/ACR Damage Index, to investigate how cumulative disease activity is related to damage in childhood‐onset SLE, and to identify other risk factors for damage in childhood‐onset SLE.

Methods

Disease activity and damage in 66 patients with newly diagnosed childhood‐onset SLE were assessed retrospectively, and information on potential risk factors for damage (age, race, sex, medications, duration of disease, hypertension, body mass index, antiphospholipid antibodies, kidney disease, acute thrombocytopenia) was obtained. In addition, a group of physicians was surveyed to establish the sensibility of the SLICC/ACR Damage Index in childhood‐onset SLE.

Results

The SLICC/ACR Damage Index was found to have face, content, and construct validity when used in children. The mean SLICC/ACR Damage Index score of the patients was 1.76 (mean followup 3.3 years). Cumulative disease activity over time was the single best predictor of damage (R² = 0.30). Other, possibly important risk factors for damage were corticosteroid treatment, the presence of antiphospholipid antibodies, and acute thrombocytopenia. It was determined that immunosuppressive agents may be protective.

Conclusion

The SLICC/ACR Damage Index, though useful in childhood‐onset SLE, may benefit from the introduction of weightings and redefinition of some of the items. Ongoing disease activity leads to disease damage, and treatment should be prompt. Prolonged use of high‐dose corticosteroids may further increase damage, but use of immunosuppressive agents may protect against disease damage; this latter finding may have potential implications for the treatment of childhood‐onset SLE and deserves further study. The relationship between disease activity and concomitant use of medication also requires further investigation.

     

Serum concentrations of aminoterminal propeptide of type III procollagen and propeptide of human type I procollagen in systemic lupus erythematosus

The purpose of this study was to assess the association between the serum levels of aminoterminal propeptide of type III procollagen (PIIINP) and carboxyterminal propeptide of type I procollagen (PICP) with disease activity and damage in systemic lupus erythematosus (SLE). Thirty-three patients with SLE were compared with 31 controls. The assessment in SLE included disease activity indices (SLEDAI, MEX-SLEDAI) and damage index (SLICC/ACR). PIIINP and PICP were measured by radioimmunoassay. Compared with controls, mean levels of PIIINP were higher in SLE (2.9±1.8 vs. 1.8±1.2, P=0.006). PICP was also increased in SLE versus controls (163±94 vs. 102±62, P=0.007). PIIINP was correlated with SLICC/ACR (r=0.33, P=0.048). No correlation was observed between PICP and PIIINP with other clinical or therapeutic variables. These preliminary data suggests a role of PIIINP as a marker for chronic damage. Follow-up studies are required to evaluate its utility in predicting future damage.     

Study of anti-apolipoprotein A-I antibodies and paraoxonase 1 activity in systemic lupus erythematosus patients; correlation with disease activity and damage indices

IntroductionSystemic lupus erythematosus (SLE) patients have an increased risk of atherosclerosis. Identification of at-risk patients and the pathogenesis of atherosclerosis in SLE remain elusive. Paraoxonase 1 (PON1) and anti-apolipoprotein A-I antibody (anti-Apo A-I) appear to have a potential role in premature atherosclerosis in SLE.Aim of the workTo assess two novel risk factors of atherosclerosis in SLE patients; PON1 activity, and anti-Apo A-I antibody levels, in order to elucidate any possible correlation between both of them, and to demonstrate their relations to disease activity disease activity as well as disease related damage.Patients and methodsForty SLE female patients and 40 apparently healthy volunteers were included in this study. Anti-Apo A-I antibody levels and PON1 activity levels were assessed. Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Systemic Lupus International Collaboration Clinics (SLICC)/American College of Rheumatology (ACR) damage index were preformed to all patients.ResultsCompared with controls, SLE patients showed significantly lower PON1 activity and significantly higher titers of anti Apo A-I. Anti-Apo A-I antibody titers correlated inversely with PON1 activity. Elevated titers of anti-Apo A-I antibody and reduced PON1 activity were related to increased SLEDAI and (SLICC/ACR) damage index scores.ConclusionThere is a decreased PON1 activity and formation of anti-Apo A-I antibodies in SLE patients and both of them correlated with disease activity as well as disease-related damage. PON1 activity and anti-Apo A-I antibodies might be involved in the pathogenesis of premature atherosclerosis in SLE patients.     

Risk factors for cataracts in a cohort of Egyptian systemic lupus erythematosus patients

Aim of the workThis study was undertaken to evaluate cataract risk factors in Egyptian systemic lupus erythematosus (SLE) patients.Patients and methodsA cohort of 150 consecutive adult SLE patients was included. None of the patients was diabetic or had cataract before steroids treatment for SLE. Detailed medical and ophthalmological histories were taken. Thorough clinical examination was performed with assessment of SLE disease activity index (SLEDAI) and systemic lupus international collaborating clinics damage index (SLICC DI), along with routine blood tests. Slit lamp examination was done to assess the anterior segment of the eye especially the lens to detect the type and degree of cataract.Results150 SLE patients were studied with a mean age of 37.16 ± 11 years; 129 females and 21 males and a mean disease duration of 11 ± 5.7 years. Their mean SLEDAI was 4.1 ± 3.78 and SLICC/DI 2.1 ± 1.3. The frequency of cataract was 18% (n = 27); 22.2% of them were cortical and 77.8% were posterior subcapsular with mild-moderate degree. Glaucoma was present in only 2%. A significant increase in age, disease duration, age at onset, SLICC/DI, cumulative steroid dose and hydroxychloroquine (HCQ) dose was found in those with cataract compared to non-cataract patients (p < 0.05). The age, age at onset, disease duration, SLICC/DI and cumulative steroid dose were significant independent factors increasing the probability of cataract occurrence among SLE patients (p < 0.01).ConclusionAlthough corticosteroids are prescribed to control SLE symptoms, these results highlight potential risks associated with their use, especially cataracts, which require careful and routine ophthalmologic examination and follow up.     

Factors associated with fatigue in patients with systemic lupus erythematosus

OBJECTIVE: To examine the relation between fatigue, disease activity, damage, and quality of life measures in patients with systemic lupus erythematosus (SLE). METHODS: Consecutive patients attending the University of Toronto Lupus Clinic were studied. Disease activity was assessed using the SLEDAI and SLAM-R and damage using the SLICC/ACR Damage index. Fatigue was measured by the Fatigue Severity Score (FSS) and health status by the SF-36 questionnaire. In all cases a tender point count was also performed. RESULTS: 81 patients were studied. Their mean (SD) age and disease duration were 43 (12.5) years and 12.7 (8.0) years respectively. The FSS did not correlate with the SLEDAI nor with the SLAM-R. There was no correlation with the SLICC damage index. Fatigue severity correlated with the tender point count (SCC r=0.46, p<0.001), and negatively with all domains of the SF36 (r values -0.50 to -0.82). Disease activity and damage accounted for only 4.8% and 4% respectively of the variance in fatigue severity reported by patients. CONCLUSION: In an outpatient population of SLE patients, fatigue severity correlates with poor health status and a higher tender point count. In patients with SLE, factors associated with quality of life and fibromyalgia seem to have a greater influence on the severity of reported fatigue than does the level of current disease activity.     

Serum levels of soluble Fas correlate with indices of organ damage in systemic lupus erythematosus

OBJECTIVE: To evaluate whether the levels of soluble form of the Fas apoptosis antigen (sCD95/sFas) varied from those of healthy control subjects in a group of patients with systemic lupus erythematosus (SLE). This was done to determine whether sFas has a role in either the disease activity or the organ damage in SLE. METHODS: Serum levels of sFas were measured over a period of 4 y (277 determinations) in 39 Arab patients with SLE and 22 age-, gender-, and race-matched healthy controls using double antibody ELISA. SLEDAI scores for disease activity and SLICC/ACR scores for cumulative organ damage were determined. Serum levels of acute phase reactants, complement, inflammatory cell counts, levels of autoantibodies, and kidney and liver function test results were obtained retrospectively from clinical records. RESULTS: sFas levels were significantly higher in patients with SLE (n = 39, 277 determinations) (0.60 ng/ml +/- 0.38) than in healthy controls (n = 22) (0.26 ng/ml +/- 0.11) (P < 0.00001). The levels of sFas correlated with SLICC/ACR (r = 0.36; P < 0.02), but not with SLEDAI. sFas correlated with renal and liver function tests measured by s-creatinine (r = 0.38; P < 0.0001), creatinine clearance (r = -0.30, P < 0.001), s-albumin (r = -0.28, P < 0.0001), and ALT (r = 0.35; P < 0.00001), but did not correlate with the levels of acute phase reactants. CONCLUSION: sFas is elevated in sera of SLE patient. Since sFas correlates with indices of organ damage but not with disease activity, it may be a marker of organ damage in SLE and may act to protect certain organs from further damage by inhibiting Fas-mediated apoptosis.     

Neuropsychiatric damage in Southern Chinese patients with systemic lupus erythematosus

We conducted the current study to determine the prevalence and predictors of neuropsychiatric damage in a cohort of Chinese patients with systemic lupus erythematosus (SLE). Patients were those newly diagnosed as having SLE between 1990 and 2004 in our unit. Demographic data, presenting and cumulative clinical features, disease activity score at diagnosis, and serial damage scores were obtained. Neuropsychiatric (NP) manifestations were classified according to the American College of Rheumatology (ACR) nomenclature. NP damage was evaluated by the NP domain of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index. Factors predictive of NP damage were studied by regression models. We studied 282 patients who fulfilled > or =4 of the ACR criteria for SLE. The mean age of SLE onset was 31.8 +/- 14 years. After a mean follow-up of 6.7 years, 65 patients (23%) had at least 1 NP manifestation and 50 (18%) developed NP damage (SLICC/ACR Damage Index > or = 1). Cerebrovascular accident was the most common cause of NP damage (35%), followed by seizure (20%), psychosis (12%), cranial/peripheral neuropathy (12%), cognitive dysfunction (12%), and myelopathy (9%). In a multiple regression model, disease activity at diagnosis, cumulative non-NP damage, presence of antiphospholipid antibodies, and ever use of pulse methylprednisolone were independent factors associated with NP damage. New NP damage after the first year of diagnosis was predicted by longer disease duration and the use of pulse methylprednisolone in another multivariate model. Neither early nor cumulative NP damage predicted mortality. NP damage is prevalent in Chinese patients with SLE and is independently associated with more active disease at diagnosis, antiphospholipid antibodies and the use of pulse methylprednisolone therapy. Primary prevention for cerebrovascular disease in high-risk patients may reduce NP damage.     

Age-related differences in the clinical characteristics of systemic lupus erythematosus in children

The objective of this study was to determine how the clinical presentation of systemic lupus erythematosus in pediatric varied with the age of onset of the disease. We reviewed the charts of a total of 88 Chinese pediatric patients (pSLE) diagnosed and admitted first time to our hospitals between 2005 and 2008. Patients were divided into 3 groups, depending on the age at diagnosis: preschool (1–6 years), school age (7–11 years), and adolescent groups (12–18 years). Among the three groups, we compared the sex ratio, disease duration at diagnosis, symptoms at the onset of the disease, clinical manifestations, laboratory examinations, SLEDAI (Systemic Lupus Erythematosus Disease Activity Index) 2000, and SLICC/ACR SDI (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index for Systemic Lupus Erythematosus). Most pediatric patients were in the adolescent group (47.7%), while the disease duration at diagnosis was shortest in preschool-age patients (0.65 ± 0.5 months). The most common symptoms at the onset of pSLE were fever, rash, arthritis, and seizures. Hematologic system and neuropsychiatric system were damaged commonly. The preschool-age patients had the shortest disease duration at diagnosis, the highest incidence of neuropsychiatric system involvement, and the poorest prognosis of all the age groups, especially the boys. Patients in adolescence had the peak incidence of pediatric SLE and high disease activity, begins to acquire some of the adult characteristics. School-age is a transition stage between other stages.     

Clinical features and long-term outcomes of systemic lupus erythematosus: comparative data of childhood,adult and late-onset disease in a national register

Systemic lupus erythematosus (SLE) affects predominantly women at reproductive age but may present at any age. Age at disease onset has a modulating effect on presentation and course of disease, but controversies persist regarding its impact on long-term outcome. Our aims were to characterize clinical features, co-morbidities and cumulative damage in childhood-onset, adult-onset and late-onset SLE. Patients with childhood-onset SLE fulfilling ACR 1997 criteria were identified in a nationwide register-Reuma.pt/SLE (N = 89) and compared with adult-onset and late-onset counterparts matched 1:1:1 for disease duration. 267 SLE patients with mean disease duration of 11.9 ± 9.3 years were analyzed. Skin (62 %), kidney (58 %), neurological (11 %) and hematologic involvement (76 %) were significantly more common in childhood-onset SLE and disease activity was higher in this subset than in adult- and late-onset disease (SLEDAI-2K 3.4 ± 3.8 vs. 2.2 ± 2.7 vs. 1.6 ± 2.8, respectively; p = 0.004). Also, more childhood-onset patients received cyclophosphamide (10 %) and mycophenolate mofetil (34 %). A greater proportion of women (96 %), prevalence of arthritis (89 %) and anti-SSA antibodies (34 %) were noted in the adult-onset group. There was a significant delay in the diagnosis of SLE in older ages. Co-morbidities such as hypertension, diabetes and thyroid disease were significantly more frequent in late-onset SLE, as well as the presence of irreversible damage evaluated by the SLICC/ACR damage index (20 vs. 26 vs. 40 %; p < 0.001). Greater organ involvement as well as the frequent need for immunosuppressants supports the concept of childhood-onset being a more severe disease. In contrast, disease onset is more indolent but co-morbidity burden and irreversible damage are greater in late-onset SLE, which may have implications for patients’ management.     

Association between clinical factors, socioeconomic status, and organ damage in recent onset systemic lupus erythematosus

OBJECTIVE: To determine the prevalence and socioeconomic and clinical predictors of early organ damage in a cohort of patients with systemic lupus erythematosus (SLE) of 2-7 years' duration randomly sampled at 5 centers and balanced by socioeconomic status and race. METHODS: The Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index was measured in 200 patients who met the ACR criteria for SLE with a mean disease duration of 3.8 years. The SLICC/ACR scores for each organ system and the prevalence of damage within organ systems were assessed. Logistic regression analyses evaluated the simultaneous effects of age at diagnosis, disease duration, disease activity, and sociodemographic factors. RESULTS: Sixty-one percent of the patients had damage within 7 years of onset (mean 3.8 yrs). Neuropsychiatric (20.5%) and musculoskeletal (18.5%) systems were the most frequently involved, followed by renal (15.5%) and skin (12.5%) systems, all with median SLICC/ACR organ system scores of 1. In multivariate models, African-American race was associated with skin damage but not with damage in other specific organ systems. Socioeconomic status was not associated with organ system damage. Older age at diagnosis correlated with cardiovascular, musculoskeletal, gastrointestinal, ocular, and pulmonary damage. Clinical factors such as longer disease duration correlated with higher renal and cardiovascular damage, and greater disease activity at diagnosis of SLE correlated with greater renal, musculoskeletal, and pulmonary damage. CONCLUSION: There is evidence of organ system damage in SLE within a mean of 3.8 years after onset. We found little evidence for differences in early organ damage according to race or socioeconomic status. Damage to most organ systems was related to age at diagnosis of SLE and clinical factors such as disease duration.