SLICC/ACR Damage Index Research Articles

Self-Reported Physical Activity Is Associated with Lupus Nephritis in Systemic Lupus Erythematosus: Data from KORean Lupus Network (KORNET) Registry.

PurposeThe aim of this study was to identify the associations among physical activity, disease activity, and organ damage in patients with systemic lupus erythematosus (SLE).Materials and MethodsA total of 415 patients with SLE were consecutively enrolled from the KORean lupus Network (KORNET) registry. This registry assessed clinical features, disease activity [Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K)], and organ damage [Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (SDI)] upon enrollment in the study. Self-reported physical activity was measured by the International Physical Activity Questionnaire. Statistical analyses were conducted using the Mann-Whitney U test and multivariate logistic regression analysis.ResultsA significant difference in vigorous activity was noted between patients with lupus nephritis (LN) (n=93) and those without LN (n=322) (p=0.012), but not in moderate and walking activities. In contrast, no differences in physical activity, walking, moderate, and vigorous intensity, according to SLEDAI-2K and SDI were found. In addition to younger age (p=0.032), high physical component summary of SF-36 (p=0.004) and SLEDAI-2K (p=0.038), and less vigorous physical activity were associated with LN (p=0.024). However, cardiovascular disease was not associated with physical activity in SLE patients.ConclusionThis study showed that patients with LN had less vigorous physical activity than patients without LN. The results suggest that lupus nephritis might be associated with physical activity.

Remission and Low Disease Activity Status (LDAS) protect lupus patients from damage occurrence: data from a multiethnic, multinational Latin American Lupus Cohort (GLADEL)

ObjectiveTo evaluate disease activity statuses’ (DAS’) impact on systemic lupus erythematosus (SLE) outcomes.Materials and methodsFour DAS were defined: remission off-therapy: SLE Disease Activity Index (SLEDAI)=0, no prednisone or immunosuppressive drugs...

Sustained complete renal remission is a predictor of reduced mortality, chronic kidney disease and end-stage renal disease in lupus nephritis.

Objective The objective of this paper is to identify the relationship between patients with lupus nephritis (LN) who achieve sustained complete renal remission (CR) and renal outcome and survival. Methods From a longitudinal cohort study we identified patients with LN with CR. We compared the outcomes of patients who achieved sustained CR for at least five years (Group A) with those less than five years (Group B). The outcomes were death, SLICC/ACR damage index (SDI), renal flare, end-stage renal disease (ESRD) or estimated glomerular filtration rate (eGFR) < 50 ml/min, and doubling of serum creatinine. Regression analyses were used to identify predictors of the outcomes. Results A total of 345 patients were identified, 132 patients in Group A and 213 patients in Group B. The duration of CR in Group A was 11.76 ± 7.34 years but only 1.24 ± 1.24 years in Group B ( p < 0.001). Death, increasing renal SDI, renal flare, renal transplantation, ESRD or eGFR < 50 ml/min, and doubling of serum creatinine in Group A were significantly lower than Group B. Multivariable analysis revealed that Group A patients were at a lower risk of death (hazard ratio (HR) = 0.20; 95% confidence interval (CI), 0.07-0.61; p = 0.004), increasing renal SDI (HR = 0.41; 95% CI, 0.21-0.76; p = 0.01), developing ESRD or eGFR < 50 ml/min (HR = 0.27; 95% CI, 0.12-0.61; p = 0.001), and doubling of serum creatinine (HR = 0.29; 95% CI, 0.14-0.61; p = 0.001) compared with Group B. Conclusion Sustained CR for at least five years is a predictor of better prognosis in patients with LN.

Osteopontin is associated with disease severity and antiphospholipid syndrome in well characterised Swedish cases of SLE

ObjectiveThe variety of disease phenotypes among patients with SLE challenges the identification of new biomarkers reflecting disease activity and/or organ damage. Osteopontin (OPN) is an extracellular matrix protein with immunomodulating...

Does pregnancy have any impact on long term damage accrual and on the outcome of lupus nephritis?

No data are available about the impact of pregnancy on the long-term outcome of lupus nephritis.Thirty-two women with lupus nephritis with a 10-year follow-up after their first pregnancy (“women who gave birth”) and 64 matched controls with the same follow-up and who never had pregnancies (“controls”) were compared for the occurrence of SLE flares, chronic kidney disease (CKD), and SLICC/ACR Damage Index (SDI) in the post pregnancy period. The same evaluations were done before and after pregnancy in women who gave birth. The predictors of CKD and damage accrual in the whole population were studied.All patients were Caucasians and had biopsy proven LN. At conception and after 10 years, in both groups, less than 10% of patients had active renal disease (p = ns). Controls had more frequent arterial hypertension (p = 0.025). Between the two groups there was no difference in SLE flares and in CKD free survival curves (p = 0.6 and p = 0.37) during the 10-year follow-up. In both groups, the temporal trend, based on annual evaluation, of glomerular filtration rate and serum creatinine shows a significant decrease and increase respectively. However, the women who gave birth persistently maintained better values of renal function than controls during the whole follow-up (P = 0.00001). There was no difference in the CKD-free survival curves. SDI did not increase significantly in women who gave birth in comparison to controls. All the above mentioned clinical parameters were comparable before and after pregnancy in the women who gave birth.Among the basal clinical characteristics, high serum creatinine and occurrence of SLE flares predicted CKD, whereas low levels of C3, pre-existing damage score, and occurrence of SLE flares predicted SDI increase. Pregnancy was not a predictor of CKD or SDI increase.Carrying a pregnancy during inactive lupus nephritis does not seem to increase the rate of SLE flares, worsen renal prognosis or increase SDI significantly in the very long-term.

Prediction of chronic damage in systemic lupus erythematosus by using machine-learning models.

ObjectiveThe increased survival in Systemic Lupus Erythematosus (SLE) patients implies the development of chronic damage, occurring in up to 50% of cases. Its prevention is a major goal in the SLE management. We aimed at predicting chronic damage in a large monocentric SLE cohort by using neural networks.MethodsWe enrolled 413 SLE patients (M/F 30/383; mean age ± SD 46.3±11.9 years; mean disease duration ± SD 174.6 ± 112.1 months). Chronic damage was assessed by the SLICC/ACR Damage Index (SDI). We applied Recurrent Neural Networks (RNNs) as a machine-learning model to predict the risk of chronic damage. The clinical data sequences registered for each patient during the follow-up were used for building and testing the RNNs.ResultsAt the first visit in the Lupus Clinic, 35.8% of patients had an SDI≥1. For the RNN model, two groups of patients were analyzed: patients with SDI = 0 at the baseline, developing damage during the follow-up (N = 38), and patients without damage (SDI = 0). We created a mathematical model with an AUC value of 0.77, able to predict damage development. A threshold value of 0.35 (sensitivity 0.74, specificity 0.76) seemed able to identify patients at risk to develop damage.ConclusionWe applied RNNs to identify a prediction model for SLE chronic damage. The use of the longitudinal data from the Sapienza Lupus Cohort, including laboratory and clinical items, resulted able to construct a mathematical model, potentially identifying patients at risk to develop damage.

Cross-cultural validation of Lupus Impact Tracker in five European clinical practice settings.

The aim was to evaluate the cross-cultural validity of the Lupus Impact Tracker (LIT) in five European countries and to assess its acceptability and feasibility from the patient and physician perspectives. A prospective, observational, cross-sectional and multicentre validation study was conducted in clinical settings. Before the visit, patients completed LIT, Short Form 36 (SF-36) and care satisfaction questionnaires. During the visit, physicians assessed disease activity [Safety of Estrogens in Lupus Erythematosus National Assessment (SELENA)-SLEDAI], organ damage [SLICC/ACR damage index (SDI)] and flare occurrence. Cross-cultural validity was assessed using the Differential Item Functioning method. Five hundred and sixty-nine SLE patients were included by 25 specialists; 91.7% were outpatients and 89.9% female, with mean age 43.5 (13.0) years. Disease profile was as follows: 18.3% experienced flares; mean SELENA-SLEDAI score 3.4 (4.5); mean SDI score 0.8 (1.4); and SF-36 mean physical and mental component summary scores: physical component summary 42.8 (10.8) and mental component summary 43.0 (12.3). Mean LIT score was 34.2 (22.3) (median: 32.5), indicating that lupus moderately impacted patients' daily life. A cultural Differential Item Functioning of negligible magnitude was detected across countries (pseudo- R 2 difference of 0.01-0.04). Differences were observed between LIT scores and Physician Global Assessment, SELENA-SLEDAI, SDI scores = 0 (P < 0.035) and absence of flares (P = 0.004). The LIT showed a strong association with SF-36 physical and social role functioning, vitality, bodily pain and mental health (P < 0.001). The LIT was well accepted by patients and physicians. It was reliable, with Cronbach α coefficients ranging from 0.89 to 0.92 among countries. The LIT is validated in the five participating European countries. The results show its reliability and cultural invariability across countries. They suggest that LIT can be used in routine clinical practice to evaluate and follow patient-reported outcomes in order to improve patient-physician interaction.

Outcomes of 847 childhood-onset systemic lupus erythematosus patients in three age groups.

Objective The objective of this study was to assess outcomes of childhood systemic lupus erythematosus (cSLE) in three different age groups evaluated at last visit: group A early-onset disease (<6 years), group B school age (≥6 and <12 years) and group C adolescent (≥12 and <18 years). Methods An observational cohort study was performed in ten pediatric rheumatology centers, including 847 cSLE patients. Results Group A had 39 (4%), B 395 (47%) and C 413 (49%). Median disease duration was significantly higher in group A compared to groups B and C (8.3 (0.1-23.4) vs 6.2 (0-17) vs 3.3 (0-14.6) years, p < 0.0001). The median Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI) (0 (0-9) vs 0 (0-6) vs 0 (0-7), p = 0.065) was comparable in the three groups. Further analysis of organ/system damage revealed that frequencies of neuropsychiatric (21% vs 10% vs 7%, p = 0.007), skin (10% vs 1% vs 3%, p = 0.002) and peripheral vascular involvements (5% vs 3% vs 0.3%, p = 0.008) were more often observed in group A compared to groups B and C. Frequencies of severe cumulative lupus manifestations such as nephritis, thrombocytopenia, and autoimmune hemolytic anemia were similar in all groups ( p > 0.05). Mortality rate was significantly higher in group A compared to groups B and C (15% vs 10% vs 6%, p = 0.028). Out of 69 deaths, 33/69 (48%) occurred within the first two years after diagnosis. Infections accounted for 54/69 (78%) of the deaths and 38/54 (70%) had concomitant disease activity. Conclusions This large multicenter study provided evidence that early-onset cSLE group had distinct outcomes. This group was characterized by higher mortality rate and neuropsychiatric/vascular/skin organ damage in spite of comparable frequencies of severe cumulative lupus manifestations. We also identified that overall death in cSLE patients was an early event mainly attributed to infection associated with disease activity.

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 6months. 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.

Severe vitamin D deficiency increases the risk for moderate to severe disease activity in Chinese patients with SLE.

We aimed to investigate the prevalence of vitamin D deficiency in Chinese lupus patients and to assess the association between vitamin D levels and disease severity. Serum levels of 25OHD3 in 121 patients with systemic lupus erythematosus (SLE) and 150 healthy controls were measured by electrochemiluminescence immunoassay. Data regarding demographics and clinical parameters were collected. Disease activity of SLE was evaluated according to the SLE Disease Activity Index (SLEDAI) score and irreversible organ damage by the Systemic Lupus International Collaborating Clinic/American College of Rheumatology, SLICC/ACR Damage Index (SDI). The multivariate logistic regression model was used to investigate the association between the degree of vitamin D deficiency and SLEDAI or SDI scores. The prevalence of vitamin D insufficiency (25OHD3 <30 ng/ml) and severe deficiency (25OHD3 <10 ng/ml) in SLE patients was 62.81% and 34.71%, respectively. Logistic regression analysis indicated that the cut-off point of 25OHD3 concentration was 10 ng/ml where its level was correlated with increased SLEDAI (OR 6.420, p = 0.006), but not with the SDI. In addition, hydroxychloroquine treatment lowered the SLEDAI increased by the severe 25OHD3 deficiency (OR 0.280, p = 0.008). Moreover, long disease duration (OR 1.014, p = 0.008) predicted moderate to severe organ damage. Vitamin D deficiency is highly prevalent in patients with SLE. Severe deficiency increases the risk for moderate to severe disease activity, but not for organ damage.

Organ Damage and Quality of Life in Antiphospholipid Syndrome.

Antiphospholipid syndrome (APS) affects young patients in the most productive years of their life, and the consequences of organic or tissue damage involve a decrease in health-related quality of life (HRQoL). While acute disease manifestations of APS are well known, information on the long-term prognosis and damage in affected patients is still very limited. Systemic lupus erythematosus (SLE) patients would be expected to experience long-term complications and even die as a consequence of APS. Organ damage in APS has been evaluated using different methods and definitions, including the SLICC/ACR Damage Index (SDI), which tend to underestimate aPL-related damage. A new damage index in APS has been proposed (DIAPS), and it seems to be more accurate than SDI. Given the implications for morbidity and mortality, it is imperative to assess accurately aPL-related damage and HRQoL in patients with APS.

Bone impairment assessed by HR-pQCT in juvenile-onset systemic lupus erythematosus.

High-resolution peripheral quantitative computed tomography (HR-pQCT) analysis of female juvenile-onset systemic lupus erythematosus (JoSLE) patients revealed trabecular/cortical bone damage and reduced bone strength primarily at the distal radius compared to healthy controls. We demonstrated for the first time that JoSLE patients with vertebral fracture (VF) present trabecular impairment at the distal radius. This study investigated the volumetric bone mineral density (vBMD), microarchitecture, and biomechanical features at the distal radius and tibia using HR-pQCT and laboratory bone markers in JoSLE patients compared to controls to determine whether this method discriminates JoSLE patients with or without VF. We compared 56 female JoSLE patients to age- and Tanner-matched healthy controls. HR-pQCT was performed at the distal radius and tibia. Serum levels of the amino-terminal pro-peptide of type I collagen, the C-terminal telopeptide of type I collagen, intact parathormone, sclerostin, and 25-hydroxyvitamin D (25OHD) were evaluated. VFs were analyzed using VFA-dual-energy X-ray absorptiometry (DXA) (Genant's method). Reduced density and strength parameters and microarchitecture alterations of cortical and trabecular bones were observed in JoSLE patients compared to controls, primarily at the distal radius (p < 0.05). Patients with VF exhibited a significant decrease in trabecular bone parameters solely at the distal radius (Total.BMD, p = 0.034; Trabecular.BMD [Tb.BMD], p = 0.034; bone volume (BV)/trabecular volume (TV), p = 0.034; apparent modulus, p = 0.039) and higher scores for disease damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI), p = 0.002). Bone metabolism markers were similar in all groups. Logistic regression analysis of parameters that were significant in univariate analysis revealed that Tb.BMD (OR 0.98, 95% CI 0.95-0.99, p = 0.039) and SLICC/ACR-DI (OR 7.37, 95% CI 1.75-30.97, p = 0.006) were independent risk factors for VF. In conclusion, this study is the first demonstration of bone microstructure and strength deficits in JoSLE patients, particularly at the distal radius. Our results demonstrated that VF was associated with trabecular radius alteration and emphasized the potential detrimental effect of disease damage on this condition.

Evaluation of left atrial function by speckle tracking echocardiography in patients with systemic lupus erythematosus.

Left atrial (LA) function plays a key role in maintaining optimal cardiac output. Left ventricular diastolic dysfunction (LVDD) has been reported in systemic lupus erythematosus (SLE), but whether LA functional abnormalities also occur in patients with SLE is unknown. Toward this aim we evaluated left atrial function and volume by strain and strain rate derived from speckle tracking echocardiography (STE) and their associations with LVDD. Sixty SLE patients were compared with age- and gender-matched normal controls. The LA strain (S) and strain rate (SR) during systole, early diastole and late diastole (SRs, SRe and SRa, respectively) were measured by STE. The LA volume index (LAVI), traditional parameters of LA and left ventricular diastolic function also were analysed. Global strain and positive SRe were significantly reduced in the SLE group compared with the control group (26.2% ± 9.5% vs 32.5% ± 9.8% and -2.4 ± 1.0 s(-1) vs -3.1 ± 1.2 s(-1), both p < 0.05). The SRs in the SLE and control groups were not significantly different (2.1 ± 0.7 s(-1) vs 2.4 ± 0.8 s(-1), p = 0.2). The positive SRa was increased in the SLE group compared with the control group (-2.1 ± 0.8 s(-1) vs -1.6 ± 0.5 s(-1), p < 0.05) and the LAVI was larger in the SLE group than in the control group (32.4 ± 8.0 vs 25.8 ± 7.1 ml/m(2), p < 0.001). Patients with SLE exhibiting varying grades of LVDD displayed significant differences in LA parameters, including LAVI, SRs, SRe and SRa (all p < 0.05). Multivariate linear analysis additionally revealed that SLICC/ACR damage index (SDI) was independently and inversely associated with global strain, SRs and positive SRe. LA functions were changed in SLE patients, demonstrating impairment in conduit function, decrease in storage function and increase in pump function. Meanwhile, the magnitude of this impairment was predictively associated with the severity of LVDD. The results from this study demonstrate that STE is capable of detecting various aspects of LA functional impairment during SLE progression, and should be further explored as a diagnostic tool for improving the outcomes of SLE patients.

Non-corticosteroid risk factors of symptomatic avascular necrosis of bone in systemic lupus erythematosus: A retrospective case-control study

Objective. Avascular necrosis of bone (AVN) is an important complication of systemic lupus erythematosus (SLE). Corticosteroid therapy has been underlined as a main risk factor for osteonecrosis. However, AVN development in patients who have never received corticosteroid and the absence of AVN in the majority of the patients, who received corticosteroid, propose a role for non-corticosteroid risk factors in AVN development.Methods. This case-control study included two subsets: oral corticosteroid (66 AVN and 248 non-AVN patients) and pulse-therapy subset (39 AVN and 312 non-AVN patients) who have attended our Lupus clinic from 1979 to 2009. Patients received similar cumulative dose corticosteroid, equal maximum dose and 1-year maximum dose of corticosteroid. The demographic data (including sex, age of disease onset, age at the diagnosis of AVN), organs involvement, SLE Disease Activity Index (SLEDAI), Systemic Lupus International Collaborating Clinics/American College of Rheumatology-Damage index (SLICC/ACR-DI), number of disease flare ups were compared between two subsets.Results. The mean age of SLE onset was younger (P value = 0.04) in the AVN patients. In oral corticosteroid subset, malar rash (P value < 0.001) and oral ulcer (P value = 0.003) were seen more frequently in non-AVN patients, whereas psychosis (P value = 0.03) was significantly more prevalent AVN subset in oral corticosteroid subset. In corticosteroid pulse subset, no significant difference in clinical features was noted.Conclusion. In oral corticosteroid subset, younger age of disease onset and psychosis were significantly associated with AVN, whereas malar rash and oral ulcer showed negative association AVN.

SEVERITY INDEX IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

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Elevated levels of serum sCXCL16 in systemic lupus erythematosus; potential involvement in cutaneous and renal manifestations.

The aim of this study was to investigate the levels and clinical significance of serum soluble chemokine (C-X-C motif) ligand 16 (sCXCL16) in patients with systemic lupus erythematosus (SLE), as well as the sCXCL16 molecule's associations with disease activity and organ damage. Thirty-five patients with SLE, 16 patients with rheumatoid arthritis (RA), and 15 healthy controls were included in this study. The demographic and clinical features of the patients were recorded. The serum levels of sCXCL16 were determined. Disease activity was assessed using the SLE Disease Activity Index (SLEDAI), and organ damage was evaluated with the Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (SDI). The serum levels of sCXCL16 in the patients with SLE were higher than those in the patients with RA (P = 0.002) or healthy controls (P < 0.0001). The levels in the patients with active SLE were higher than those in the disease inactive patients (P = 0.008). Positive correlations were identified between serum sCXCL16 concentrations and both SLEDAI (r = 0.564; P < 0.0001) and SDI scores (r = 0.396; P = 0.018). Both SLEDAI (P = 0.021) and serum levels of CXCL16 (P = 0.023) decreased after conventional treatment in 12 initial onset cases of SLE patients. Elevated serum sCXCL16 levels were discovered in the SLE patients with cutaneous (P = 0.006) and renal involvement (P = 0.032). Soluble CXCL16 may become a useful serological marker of disease activity and skin and renal involvement in SLE patients; thus, it may be used for evaluation of therapeutic interventions.

FRI0425 Prevalence of Circulating Naive CD4+ T-Cells and Memory/Naive CD4+ T-Cells Ratio Are Independently Associated with the Metabolic Syndrome (METS) in Systemic Lupus Erythematosus (SLE) Patients

BackgroundChronic immune activation may induce an increase of memory CD4+ T-cells and a decrease of naive CD4+ T-cells. In SLE patients this chronic adaptive immune activation profile could induce cardiovascular...

AB0529 Prevalence of Circulating CD4+Cd28null T-Cells is Independently Associated with Disease Damage in Systemic Lupus Erythematosus (SLE) Patients

BackgroundCD4+CD28null T-cells are a subset of long-lived cytotoxic CD4+ T-cell with pro-inflammatory functions. This subset is supposed to be caused by chronic immune stimulation by either autoantigens or not. CD4+CD28null...

Factors associated with damage accrual in patients with systemic lupus erythematosus: results from the Systemic Lupus International Collaborating Clinics (SLICC) Inception Cohort

Background and aimsWe studied damage accrual and factors determining development and progression of damage in an international cohort of systemic lupus erythematosus (SLE) patients.MethodsThe Systemic Lupus International Collaborating Clinics (SLICC)...

The number of flares patients experience impacts on damage accrual in systemic lupus erythematosus: data from a multiethnic Latin American cohort

PurposeTo determine the association between the number of flares systemic lupus erythematosus (SLE) patients experience and damage accrual, independently of other known risk factors.MethodsSLE patients (34 centres, nine Latin American...