scholarly journals Clinical profile of systemic lupus erythematosus among children less than 12 years

2018 ◽  
Vol 5 (2) ◽  
pp. 343
Author(s):  
Senthil Kumar Andy ◽  
Elayaraja Kandasamy
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Damage Index ◽  
Organ Damage ◽  
Clinical Profile ◽  
Connective Tissues ◽  
Systemic Lupus ◽  
Initial Manifestation ◽  
Double Stranded Dna

Background: Systemic lupus erythematosus (SLE) is an episodic multisystem autoimmune disease characterized by widespread inflammation of blood vessels and connective tissues and by the presence of antinuclear antibodies especially antibodies to native double stranded DNA. The aim was to study the clinical profile of SLE among children less than 12 years attending an urban referral hospital, SLEDAI scoring at onset and follow up at 1 year and SLICC/ACR damage index at 1 year.Methods: A descriptive, prospective and observational study was conducted in Medical, Nephrology, Rheumatology OPD wards, in ICH and HC, from November 2007 to August 2009 among all children diagnosed to have SLE. Results: In this study there were 50 cases over the last 2 years. Majority of the children were diagnosed within a year of their initial manifestation. The mean age at the time of onset of symptoms was 7.94 years. Female to male ratio in our study is 2.5:1.Conclusions: Efforts should be directed in diagnosing at earlier stage itself for better outcome. SLEDAI and SLICC/ACR DI can be incorporated in routine follow up to detect mild to moderate and severe flare and extent of organ damage.

SAT0179 ADHERENCE TO HYDROXYCHLOROQUINE INFLUENCES THE INCIDENCE OF ORGAN DAMAGE DURING FOLLOW-UP IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1031.1-1031
Author(s):  
J. H. Kang ◽  
S. E. Choi ◽  
H. Xu ◽  
D. J. Park ◽  
S. S. Lee
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Damage Index ◽  
Clinical Manifestations ◽  
Medication Possession Ratio ◽  
Organ Damage ◽  
Systemic Lupus ◽  
Laboratory Findings ◽  
The Impact

Background:Objectives:Hydroxychloroquine (HCQ) is a cornerstone drug in patients with systemic lupus erythematosus (SLE), decreasing the risk of flares and comorbidities and improving survival. This study investigated the effects of HCQ adherence on clinical manifestations, disease activity, and organ damage in Korean patients with SLE.Methods:Data on 299 SLE patients were obtained from the Korean Lupus Network registry. Demographic variables, clinical manifestations, laboratory findings, PGA, and SLEDAI-2000 and SLICC damage index scores were recorded at the time of enrollment and repeated annually for 4 consecutive years. Patients were divided into two groups according to the level of HCQ adherence. Adherence was defined by the medication possession ratio and dichotomized as ≤ 80% vs. > 80%. Univariate and multivariate analyses were performed to assess the impact of adherence to HCQ on clinical outcomes.Results:Of the 299 patients, 31 (10.4%) showed poor drug adherence during the follow-up period. Patients with poor HCQ adherence were older (P=0.011), less insured (P=0.024), experienced lower employment (P=0.033), and had a higher rate of comorbidities such as hypertension (P=0.048) and depression (P<0.001). The non-adherent group had higher mean and changed SLICC damage index scores than the adherent group across all 4 years. In the multivariate analysis, HCQ non-adherence was significantly associated with older age (OR, 1.043; 95% CI, 1.006–1.081; P=0.021), depression (OR, 11.98; 95% CI, 1.099–130.6; P=0.042), and an annual increase in the SLICC damage index score (OR, 2.275; 95% CI, 1.369–3.779; P=0.002).Conclusion:HCQ adherence might be influenced by age and depressive mood. Additionally, the poor adherence to HCQ in SLE patients was correlated with higher cumulative organ damage. Therefore, patients with SLE should be educated to take HCQ appropriately to improve their clinical outcome in clinical practice.Disclosure of Interests:None declared

scholarly journals FRI0146 SLICC/ACR DAMAGE INDEX IN PATIENTS WITH ANTIPHOSPHOLIPID SYNDROME WITH AND WITHOUT SYSTEMIC LUPUS ERYTHEMATOSUS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 657.2-657
Author(s):  
F. Cheldieva ◽  
T. Reshetnyak ◽  
A. Shumilova ◽  
N. Kosheleva ◽  
T. Lisitsyna ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Independent Predictor ◽  
Damage Index ◽  
Organ Damage ◽  
Systemic Lupus ◽  
Group Iii ◽  
Group I ◽  
Group Ii

Background:The role of antiphospholipid syndrome (APS) as a cause of chronic organ damage in patients with systemic lupus erythematosus (SLE) is important. While acute disease manifestations of APS are well known, information on the long-term prognosis and damage in affected patients is still very limited.Objectives:Тo assess the severity of organ damage in patients with APS without SLE and with concomitant SLE, as well as the feature of irreversible damage to internal organs in patients with primary APS during a dynamic observation using SLICC/ACR Damage Index (SDI).Methods:Тhe study included 195 patients (41 men and 154 women) who were observed at the Institute of Rheumatology from 2007 to June 2018 with a diagnosis of SLE and APS. The study inclusion criteria were: a follow-up period of at least 3 years with the study of serological markers of APS for previous years, the possibility of dynamic monitoring of patients, patient consent. Patients were divided into 3 groups, depending on presence of APS: group I -SLE with APS (n=99 with average age 34.6 [25-44), group II - SLE without APS (n=45; 33.5[26-42]) and group III - 45 (average age 37.7 [27-46]) patients with primary APS (PAPS) diagnosed according to international diagnostic criteria, without signs of any disease. In all three groups organ damage was assessed using SDI. SDI 1-2 points corresponded to moderate damage, more than 2 points - to severe.Results:A linear increase in irreversible organ damage was noted over 10 years of follow-up. At the time of inclusion in the study, the average SDI was significantly higher in the SLE + APS group than in the SLE group: 1.32 versus 0 when included (p <0.0001). A direct correlation was found between the age of patients and the value of SDI both in the group I (p=0.004, r=0.284) and in the group II (p=0.04, r=0.281) when included in the study. There was a direct correlation between the activity of the disease on the SLEDAI at the end of the study and the value of SDI (p=0.03, r=0.41) in the group II. The number of patients by the 10th year of follow-up remained in group I were 44 of 99, in group II - 24 of 51 and in group III - 14 of 45. SDI was more than 2 points in 39 (89%) of 44 patients in group I, in 12 (50%) of 24 in group II and 9 (65%) of 14 in Group III (p=0.0002 OR=13; 95% CI 2,4-70). 5 years after the start of the observation average SDI in group I and II was 2.5 and 1.3 (p <0.0001) and after 10 years - 2.8 and 1.9 (p = 0.0008) accordingly. An increase of total SDI occurred primarily due to damage to peripheral vessels due to the large number of thromboses (in 55% of patients) in the group II. Using step-by-step multiple logistic regressions in the study groups only APS was an independent predictor of increased SDI. The most common cause of an increase in SDI in the PAPS group was damage to peripheral vessels (64%) as a result of a high frequency of venous thrombosis, followed by damage to the neuropsychiatric system (55%) and the cardiovascular system (40%).Conclusion:APS is an independent predictor of increased SDI. The determination of irreversible organ damage in patients with PAPS using SDI allows us to assess the functional disorders of organs and systems and can be used in clinical practice in these patients.Disclosure of Interests:None declared

Characteristics and safety of tattoos in patients with systemic lupus erythematosus

Lupus ◽  
2019 ◽  
Vol 28 (10) ◽  
pp. 1250-1254 ◽  
Author(s):  
J M Sabio ◽  
S De Betolaza ◽  
J A Vargas-Hitos
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Matched Control ◽  
Damage Index ◽  
Organ Damage ◽  
Interquartile Range ◽  
Control Group ◽  
Systemic Lupus ◽  
Acute Complications

Objective The objective of this study was to determine the safety of tattoos in patients with systemic lupus erythematosus (SLE). Methods Patients ( N = 147; ≤55 years; 92% women) were asked if they had tattoos. The characteristics of the tattoos and the immediate complications were investigated and compared with those of a matched control group. We examined retrospectively after the tattoo was completed whether there had been flare-ups or increased organ damage (Systemic Lupus International Collaborating Clinics/American Collage of Rheumatology Damage Index (SDI)). Finally, we compared the SLE-related characteristics of patients with and without tattoos. Results Twenty-eight patients (19%, 26 women, median (interquartile range) age 33 (25–42) years, 65 tattoos in total) had ≥1 tattoo. At the time the tattoo was done the median (interquartile range) SLEDAI and SDI were 2 (0–2) and 0 (0–1), respectively. The characteristics of the tattoos were similar to those of controls. No patients experienced acute complications. After a median follow-up of 17 (12–20) months (3 (2–4) visits/year) four patients had five mild-to-moderate flare-ups. The median time between the tattoo and the flare-up was 9 (6–14) months. No increase in SDI was observed. The SLE-related characteristics of patients with and without tattoos were similar. Conclusion Tattoos seem to be safe in SLE patients with inactive or low active disease.

scholarly journals SAT0232 PERCEPTION OF THE DISEASE IN PATIENTS WITH EARLY SYSTEMIC LUPUS ERYTHEMATOSUS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1059.3-1059
Author(s):  
M. Garabajiu ◽  
L. Mazur-Nicorici ◽  
T. Rotaru ◽  
V. Salaru ◽  
S. B. Victoria ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Activity Index ◽  
Damage Index ◽  
Organ Damage ◽  
Systemic Lupus

Background:Systemic lupus erythematosus is an autoimmune disease with a major impact on patient’s quality of life.Objectives:To evaluate patient’s attitude toward early disease and factors that influence it.Methods:Performed case-control study included SLE patients that fulfilled SLICC, 2012 classification criteria. The research included two groups of patients: early SLE – 1stgroup (disease duration ≤24 months) and non-early SLE – 2ndgroup control (disease duration >24 months). The pattern of the disease activity was assessed by patient global assessment (PGA), Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and Systemic Lupus Activity Measure (SLAM), for SLE activity, SLICC/ACR Damage Index (DI) for disease irreversible changes and SF-8 for the Quality of Life (QoL).Results:A total of 101 SLE patients with 34 in the 1stgroup (early SLE) and 67 in the 2ndgroup (non-early SLE) was analyzed. The disease activity showed high disease activity in both groups by SLEDAI (7,02±4,16 and 6,26±4,43 points, p>0,05) and SLAM (7,47±4,40 and 7,31±4,10 points, p>0,05) such as (46,97±19,39 vs 47,98±22,41 points). The QoL was appreciated as low, by both components (mental and physical), in groups. The damage index was higher in the 2nd group (0,23±0,43 and 1,07±1,29, p<0,001), which can be explained by the development of irreversible changes with the increase of disease duration.The PGA in early SLE was influenced by subjective symptoms contained in SLAM index (r=0,48, p<0,05), such as fatigue and depression, and the level of the quality of life (r=0,65, p<0,001). Meantime, PGA in patients with longer disease duration (>2 years), was influenced by the presence of organ damage by SLICC/ACR DI (0,23, p<0,05) and objective findings of the disease activity contained in SLEDAI (r=0,33, p<0,005) and SLAM (0,44, p<0,001).Conclusion:The disease recognition in patients with early SLE was determined by subjective and psycho-emotional signs, while in patients with longer disease duration it was influenced by organ damage and complications.References:no referencesDisclosure of Interests:None declared

scholarly journals Association between organ damage and mortality in systemic lupus erythematosus: a systematic review and meta-analysis

BMJ Open ◽  
2020 ◽  
Vol 10 (5) ◽  
pp. e031850 ◽  
Author(s):  
Irene B Murimi-Worstell ◽  
Dora H Lin ◽  
Henk Nab ◽  
Hong J Kan ◽  
Oluwadamilola Onasanya ◽  
...  
Keyword(s):  
Systematic Review ◽  
Systemic Lupus Erythematosus ◽  
Latin American ◽  
Lupus Erythematosus ◽  
Meta Analysis ◽  
Damage Index ◽  
Organ Damage ◽  
Cochrane Library ◽  
Systemic Lupus ◽  
Cross Sectional

ObjectiveAt least half of patients with systemic lupus erythematosus (SLE) develop organ damage as a consequence of autoimmune disease or long-term therapeutic steroid use. This study synthesised evidence on the association between organ damage and mortality in patients with SLE.DesignSystematic review and meta-analysis.MethodsElectronic searches were performed in PubMed, Embase, Cochrane Library and Latin American and Caribbean Health Sciences Literature for observational (cohort, case-control and cross-sectional) studies published between January 2000 and February 2017. Included studies reported HRs or ORs on the association between organ damage (measured by the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) score) and mortality. Study quality was assessed using the modified Newcastle-Ottawa assessment. Pooled HRs were obtained using the DerSimonian and Laird random-effects model. Heterogeneity was assessed using the Cochrane Q (Q) and I2 statistics.ResultsThe search yielded 10 420 articles, from which 21 longitudinal studies were selected. Most studies (85%) were of high quality. For 10 studies evaluating organ damage (SDI) as a continuous variable and reporting HR as a measure of association, a 1-unit increase in SDI was associated with increased mortality; pooled HR was 1.34 (95% CI: 1.24 to 1.44, p<0.001; Q p=0.027, I2=52.1%). Exclusion of one potential outlying study reduced heterogeneity with minimal impact on pooled HR (1.33 (95% CI: 1.25 to 1.42), p<0.001, Q p=0.087, I2=42.0%). The 11 remaining studies, although they could not be aggregated because of their varying patient populations and analyses, consistently demonstrated that greater SDI was associated with increased mortality.ConclusionsOrgan damage in SLE is consistently associated with increased mortality across studies from various countries. Modifying the disease course with effective therapies and steroid-sparing regimens may reduce organ damage, improve outcomes and decrease mortality for patients with SLE.

scholarly journals Serum uric acid is associated with damage in patients with systemic lupus erythematosus

2020 ◽  
Vol 7 (1) ◽  
pp. e000366 ◽  
Author(s):  
Claudia Elera-Fitzcarrald ◽  
Cristina Reátegui-Sokolova ◽  
Rocio Violeta Gamboa-Cardenas ◽  
Mariela Medina ◽  
Francisco Zevallos ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Uric Acid ◽  
Serum Uric Acid ◽  
Lupus Erythematosus ◽  
Cox Regression ◽  
Activity Index ◽  
Damage Index ◽  
Systemic Lupus ◽  
The Impact

IntroductionSerum uric acid levels have been reported as predictors of cardiovascular, pulmonary, neurological and renal morbidity in patients with SLE. However, their role in cumulative global damage in these patients has not yet been determined.ObjectiveTo determine whether serum uric acid levels are associated with new damage in patients with SLE.MethodsThis is a longitudinal study of patients with SLE from the Almenara Lupus Cohort, which began in 2012. At each visit, demographic and clinical characteristics were evaluated, such as activity (Systemic Lupus Erythematosus Disease Activity Index-2K or SLEDAI-2K) and cumulative damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index or SDI). Treatment (glucocorticoids, immunosuppressive drugs and antimalarials) was also recorded. Univariable and multivariable Cox regression models were used to determine the impact of serum uric acid levels on the risk of new damage.ResultsWe evaluated 237 patients, with a mean age (SD) at diagnosis of 35.9 (13.1) years; 220 patients (92.8%) were women, and the duration of the disease was 7.3 (6.6) years. The mean SLEDAI-2K and SDI scores were 5.1 (4.2) and 0.9 (1.3), respectively. Serum uric acid level was 4.5 (1.4) mg/dL. Follow-up time was 3.1 (1.3) years, and 112 (47.3%) patients accrued damage during follow-up. In univariable and multivariable analyses, serum uric acid levels were associated with new damage (HR=1.141 (95% CI 1.016 to 1.282), p=0.026; HR=1.189 (95% CI 1.025 to 1.378), p=0.022, respectively).ConclusionHigher serum uric acid levels are associated with global damage in patients with SLE.

scholarly journals Organ damage in patients treated with belimumab versus standard of care: a propensity score-matched comparative analysis

2019 ◽  
Vol 78 (3) ◽  
pp. 372-379 ◽  
Author(s):  
Murray B Urowitz ◽  
Robert L Ohsfeldt ◽  
Ronald C Wielage ◽  
Kari A Kelton ◽  
Yumi Asukai ◽  
...  
Keyword(s):  
Propensity Score ◽  
Lupus Erythematosus ◽  
Damage Index ◽  
Standard Of Care ◽  
Organ Damage ◽  
Systemic Lupus ◽  
American College Of Rheumatology ◽  
Damage Progression

ObjectivesThe study (206347) compared organ damage progression in patients with systemic lupus erythematosus (SLE) who received belimumab in the BLISS long-term extension (LTE) study with propensity score (PS)-matched patients treated with standard of care (SoC) from the Toronto Lupus Cohort (TLC).MethodsA systematic literature review identified 17 known predictors of organ damage to calculate a PS for each patient. Patients from the BLISS LTE and the TLC were PS matched posthoc 1:1 based on their PS (±calliper). The primary endpoint was difference in change in Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) score from baseline to 5 years.ResultsFor the 5- year analysis, of 567 (BLISS LTE n=195; TLC n=372) patients, 99 from each cohort were 1:1 PS matched. Change in SDI score at Year 5 was significantly lower for patients treated with belimumab compared with SoC (−0.434; 95% CI –0.667 to –0.201; p<0.001). For the time to organ damage progression analysis (≥1 year follow-up), the sample included 965 (BLISS LTE n=259; TLC n=706) patients, of whom 179 from each cohort were PS-matched. Patients receiving belimumab were 61% less likely to progress to a higher SDI score over any given year compared with patients treated with SoC (HR 0.391; 95% CI 0.253 to 0.605; p<0.001). Among the SDI score increases, the proportion of increases ≥2 was greater in the SoC group compared with the belimumab group.ConclusionsPS-matched patients receiving belimumab had significantly less organ damage progression compared with patients receiving SoC.

scholarly journals Molecular pathways in patients with systemic lupus erythematosus revealed by gene-centred DNA sequencing

2020 ◽  
Vol 80 (1) ◽  
pp. 109-117
Author(s):  
Johanna K Sandling ◽  
Pascal Pucholt ◽  
Lina Hultin Rosenberg ◽  
Fabiana H G Farias ◽  
Sergey V Kozyrev ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Cell Receptor ◽  
Organ Damage ◽  
Risk Scores ◽  
Systemic Lupus ◽  
Receptor Signalling ◽  
Association Testing

ObjectivesSystemic lupus erythematosus (SLE) is an autoimmune disease with extensive heterogeneity in disease presentation between patients, which is likely due to an underlying molecular diversity. Here, we aimed at elucidating the genetic aetiology of SLE from the immunity pathway level to the single variant level, and stratify patients with SLE into distinguishable molecular subgroups, which could inform treatment choices in SLE.MethodsWe undertook a pathway-centred approach, using sequencing of immunological pathway genes. Altogether 1832 candidate genes were analysed in 958 Swedish patients with SLE and 1026 healthy individuals. Aggregate and single variant association testing was performed, and we generated pathway polygenic risk scores (PRS).ResultsWe identified two main independent pathways involved in SLE susceptibility: T lymphocyte differentiation and innate immunity, characterised by HLA and interferon, respectively. Pathway PRS defined pathways in individual patients, who on average were positive for seven pathways. We found that SLE organ damage was more pronounced in patients positive for the T or B cell receptor signalling pathways. Further, pathway PRS-based clustering allowed stratification of patients into four groups with different risk score profiles. Studying sets of genes with priors for involvement in SLE, we observed an aggregate common variant contribution to SLE at genes previously reported for monogenic SLE as well as at interferonopathy genes.ConclusionsOur results show that pathway risk scores have the potential to stratify patients with SLE beyond clinical manifestations into molecular subsets, which may have implications for clinical follow-up and therapy selection.

Obesity increases the incidence of new-onset lupus nephritis and organ damage during follow-up in patients with systemic lupus erythematosus

Lupus ◽  
2020 ◽  
Vol 29 (6) ◽  
pp. 578-586 ◽  
Author(s):  
Ji-Hyoun Kang ◽  
Haimuzi Xu ◽  
Sung-Eun Choi ◽  
Dong-Jin Park ◽  
Jung-Kil Lee ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Confidence Interval ◽  
Lupus Erythematosus ◽  
Odds Ratio ◽  
Global Assessment ◽  
Clinical Manifestations ◽  
Organ Damage ◽  
Physician Global Assessment ◽  
Systemic Lupus

Objective This study explored the effects of obesity on clinical manifestations, disease activity and organ damage in Korean patients with systemic lupus erythematosus (SLE). Methods We assessed 393 SLE patients annually for three consecutive years based on demographic information, clinical manifestations, laboratory findings and Physician Global Assessment, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)-2000 and Systemic Lupus International Collaborating Clinics (SLICC) damage index (SDI) scores. Patients were grouped by body mass index (BMI): normal weight, BMI <23 kg/m2; overweight, 23 kg/m2 ≤BMI <25 kg/m2; obese, BMI ≥25 kg/m2. The impact of obesity on clinical outcomes was assessed using univariate and multivariate analyses. Results Of the 393 patients, 59 (15.0%) were obese at enrollment. They had more comorbidities compared with non-obese patients, including diabetes, hypertension, hyperlipidemia and pulmonary hypertension. Nephritis at enrollment and newly developed nephritis during follow-up were more common ( p = 0.002 and p = 0.002, respectively) and Physician Global Assessment and SDI scores were higher in these patients for three consecutive years ( p = 0.017 and p = 0.039, respectively). Multivariate analysis revealed that obesity was significantly associated with development of nephritis during follow-up (odds ratio = 26.636; 95% confidence interval, 11.370–62.399; p < 0.001) and cumulative organ damage (odds ratio = 4.096; 95% confidence interval, 2.125–7.894, p < 0.001). Conclusions The incidences of newly developed nephritis and cumulative organ damage were higher in obese SLE patients than in non-obese SLE patients.

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