Mitochondrial DNA damage is associated with damage accrual and disease duration in patients with Systemic Lupus Erythematosus

Background:Patients with systemic lupus erythematosus (SLE) often require hospitalization. The cause of admission may vary, but active disease and infection are consistently reported as the main reasons for hospitalization and are associated with worse survival and damage accrual. Recent improvements in the standard of care, including minimization of glucocorticoid dose and more effective and safe immunosuppressive regimens, may have changed the incidence and risk factors for hospitalization due to these causes. Hence, it is useful to identify predictors of hospitalization to further reduce the risk of admission for disease activity and severe infection in patients with SLE.Objectives:To identify predictors of hospitalization in patients with SLE, according to the underlying cause.Methods:Patients with SLE fulfilling classification criteria (ACR’97 and/or SLICC), regularly followed at an academic lupus clinic from January 2009 to December 2020 and with at least two outpatient visits were included in this cohort study. Time to first hospitalization up to 120 months was identified separately for the following admission causes: (a) any cause; (b) active SLE; (c) infection. Predictors of hospitalization were sought through survival analysis, with distinct models for each of the major admission causes. Univariate analysis was performed using Kaplan-Meier curves and Log-Rank tests. Tested variables assessed at baseline included: gender; age at SLE onset; age; disease duration; SLE Disease Activity Index (SLEDAI-2K) score; ongoing antimalarial use; ongoing immunosuppressants; ongoing prednisolone daily dose; lupus nephritis up to baseline; SLICC Damage Index (SDI) score. Variables with p<0.1 were further tested in multivariate Cox regression models. Hazard ratios (HR) were determined with 95% confidence intervals (95%CI).Results:We included 398 patients (female: 86.2%, mean age: 41.2±15.1 years, mean disease duration: 10.1±9.2 years; previous lupus nephritis: 28.9%; mean SLEDAI-2K score: 3.4±2.7; ongoing antimalarials: 78.9%; ongoing immunosuppressant: 29.9%; ongoing prednisolone >7.5 mg/day: 17.1%; SDI score ≥1: 28.4%). During the follow-up period, 50.5%, 23.6% and 17.3% were hospitalized at least once for any cause, active SLE or infection, respectively.In the multivariate model, significant baseline predictors for hospitalization due to active disease were (table 1): SLEDAI-2K score >5; disease duration ≤2 years; ongoing immunosuppressants; SDI score ≥1. Baseline independent predictors of hospitalization for infection included (table 1): male gender; SDI score ≥1; ongoing antimalarials were protective.Table 1.Predictors of hospitalization in multivariate Cox regression according to the admission causePredictorsHospitalization for active SLEHospitalization for infectionSLEDAI-2K score >52.43 (1.53-3.88)n.s.SLE duration ≤2 years1.70 (1.04-2.77)n.s.Ongoing immunosuppressant1.91 (1.24-2.95)n.s.SDI score ≥11.82 (1.16-2.86)2.14 (1.33-3.45)Male gendern.s.2.19 (1.23-3.89)No antimalarial treatmentn.s.2.20 (1.34-3.60)Risk for each predictor reported as Hazard Ratio (95% Confidence Interval); n.s.: non-significantConclusion:Tight control of disease activity, prevention of damage accrual, and treatment with antimalarials may contribute to minimize the risk of hospitalization for these two major causes of admission in patients with SLE.Disclosure of Interests:None declared

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SAT0237 THE SYSTEMIC LUPUS INTERNATIONAL COLLABORATING CLINICS (SLICC) FRAILTY INDEX (SLICC-FI) PREDICTS DAMAGE ACCRUAL IN SYSTEMIC LUPUS ERYTHEMATOSUS (SLE) PATIENTS. DATA FROM A LATIN AMERICAN MESTIZO COHORT

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.3997 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1061.1-1062

Author(s):

M. F. Ugarte-Gil ◽

R. V. Gamboa Cárdenas ◽

C. Reategui Sokolova ◽

V. Pimentel-Quiroz ◽

M. Medina Chinchon ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Latin American ◽

Lupus Erythematosus ◽

Disease Duration ◽

Negative Binomial ◽

Frailty Index ◽

Systemic Lupus ◽

Research Support ◽

Damage Accrual

Background:The Systemic Lupus International Collaborating Clinics (SLICC) Frailty Index (SLICC-FI) has been developed as a predictor of outcomes in SLE patients1-3. However, it needs to be validated in several populations.Objectives:To evaluate the SLICC-FI as a predictor of future damage accrual in systemic lupus erythematosus (SLE) patients.Methods:Patients from a single-center prevalent cohort were included. Damage accrual was defined as the increase in SLICC/American College of Rheumatology (ACR) damage index (SDI) scores between the baseline and last visits. The SLICC-FI was measured at baseline. Univariable and multivariable negative binomial regression were performed to determine the association between the baseline SLICC-FI (per 0.05 increase) and damage accrual during follow-up, adjusted for sex, age at diagnosis, socioeconomic status, disease duration, SLE Disease Activity Index 2000 (SLEDAI-2K), SDI, prednisone daily dose, antimalarial and immunosuppressive drug use at baseline, and duration of follow-up.Results:Of the 265 patients included, 248 (93.6%) were female with mean (SD) age 35.1 (13.6) years at diagnosis. At baseline, mean (SD) SLE disease duration was 7.3 (6.5) years, SDI was 1.1 (1.3) and SLEDAI-2K was 5.3 (4.6). The mean (SD) baseline SLICC-FI was 0.22 (0.05). After a mean (SD) of 5.2 (2.2) years of follow-up, the SDI increased in 126 (47.5%) patients, and the final mean (SD) SDI score was 1.7 (1.7). Higher SLICC-FI scores at baseline predicted greater damage accrual in the univariable analysis [Incidence Rate Ratio (IRR)=1.283, (CI95% 1.072-1.536); p=0.007]. The SLICC-FI remained associated with damage accrual in the multivariable model, after adjustment for possible confounders [IRR= 1.224 (CI95% 1.007-1.488); p=0.042].Conclusion:The SLICC-FI predicts damage accrual in prevalent SLE, supporting the relevance of this index in the evaluation of SLE patients. This is the first study validating the SLICC-FI in South American populationReferences:[1]Legge A, Kirkland S, Rockwood K, et al. Construction of a Frailty Index as a Novel Health Measure in Systemic Lupus Erythematosus. J Rheumatol. 2020; 47: 72-81[2]Legge A, Kirkland S, Rockwood K, et al. Evaluating the Properties of a Frailty Index and Its Association With Mortality Risk Among Patients With Systemic Lupus Erythematosus. Arthritis Rheumatol. 2019; 71: 1297-107[3]Legge A, Kirkland S, Rockwood K, et al. Prediction of Damage Accrual in Systemic Lupus Erythematosus Using the Systemic Lupus International Collaborating Clinics Frailty Index (SLICC-FI). Arthitis Rheumatol. Epub ahead of print 2019 Oct 21.Disclosure of Interests:Manuel F. Ugarte-Gil Grant/research support from: Jannsen, Pfizer, Rocío Violeta Gamboa Cárdenas Grant/research support from: Pfizer, Cristina Reategui Sokolova: None declared, Victor Pimentel-Quiroz: None declared, Mariela Medina Chinchon: None declared, Claudia Elera-Fitzcarrald Consultant of: Tecnofarma, Jose Alfaro Lozano Speakers bureau: Lilly, Zoila Rodriguez Bellido: None declared, Cesar Pastor Asurza: None declared, Risto Perich Campos Consultant of: Pfizer, Speakers bureau: Pfizer, Graciela S Alarcon: None declared

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The Main Challenges in Systemic Lupus Erythematosus: Where Do We Stand?

Journal of Clinical Medicine ◽

10.3390/jcm10020243 ◽

2021 ◽

Vol 10 (2) ◽

pp. 243

Author(s):

Matteo Piga ◽

Laurent Arnaud

Keyword(s):

Systemic Lupus Erythematosus ◽

Disease Activity ◽

Lupus Erythematosus ◽

Systemic Disease ◽

Unmet Need ◽

Clinical Manifestations ◽

New Drugs ◽

Systemic Lupus ◽

Major Step ◽

Damage Accrual

Systemic lupus erythematosus (SLE) is an immune-mediated multi-systemic disease characterized by a wide variability of clinical manifestations and a course frequently subject to unpredictable flares. Despite significant advances in the understanding of the pathophysiology and optimization of medical care, patients with SLE still have significant mortality and carry a risk of progressive organ damage accrual and reduced health-related quality of life. New tools allow earlier classification of SLE, whereas tailored early intervention and treatment strategies targeted to clinical remission or low disease activity could offer the opportunity to reduce damage, thus improving long-term outcomes. Nevertheless, the early diagnosis of SLE is still an unmet need for many patients. Further disentangling the SLE susceptibility and complex pathogenesis will allow to identify more accurate biomarkers and implement new ways to measure disease activity. This could represent a major step forward to find new trials modalities for developing new drugs, optimizing the use of currently available therapeutics and minimizing glucocorticoids. Preventing and treating comorbidities in SLE, improving the management of hard-to-treat manifestations including management of SLE during pregnancy are among the remaining major unmet needs. This review provides insights and a research agenda for the main challenges in SLE.

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POS0712 “EXTRA”- CRITERIA ANTIPHOSPHOLIPID ANTIBODIES IN PATIENTS WITH ANTIPHOSPHOLIPID SYNDROME AND SYSTEMIC LUPUS ERYTHEMATOSUS (PRELIMINARY DATA)

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.818 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 605.2-606

Author(s):

F. Cheldieva ◽

T. Reshetnyak ◽

M. Cherkasova ◽

N. Seredavkina ◽

A. Lila

Keyword(s):

Systemic Lupus Erythematosus ◽

Antiphospholipid Syndrome ◽

Lupus Erythematosus ◽

Preliminary Data ◽

Antiphospholipid Antibodies ◽

Disease Duration ◽

Past History ◽

Igg Antibodies ◽

Systemic Lupus ◽

Pregnancy Morbidity

Background:The study of antiphospholipid antibodies (aPL), not included in the Sydney diagnostic criteria, in antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE) is poorly understood.Objectives:To determine the frequency of detection of IgA-aCL and IgA-aβ2GP1 and IgG antibodies to β2GP1 domain 1 (IgG-aβ2GP1-D1) in patients with APS with and without SLE.Methods:ELISA and chemiluminescence assays (CMA) were used to test 63 sera of patients: 22 (35%) with primary APS (pAPS) and 41 (65%) patients with APS and with SLE (secondary APS (sAPS)), with mean age 38,0 [33,0 – 43,0] years and disease duration 4,0 [0,1 – 9,9]. Both methods were used to test of IgG/IgM-aCL and IgG/IgM-aβ2GP1. CMA was used for research IgG/IgM/IgA-aCL, IgG/IgM/IgA-aβ2GPI and IgG-aβ2GP1-D1. Of them 49 (78%) (18 – with pAPS; 31 – with sAPS) displayed major thrombotic events and 18 of 22 pregnant women had pregnancy morbidity in past history. Lupus anticoagulant (LA) positivity was in 9 out of 12 patients who had it determined. LA was not investigated due to anticoagulant therapy in the remaining 52 patients.Results:IgG/IgM-aCL and IgG/IgM-aß2GP1 were recorded in 44/18 and 50/17 patients by ELISA and in 55/19 and 59/16 by CMA, respectively.IgA-aCL positivity was found in 35 (56%) of 63 patients. Thirty IgA-positive patients were positive for IgG-aCL by ELISA: 22 – IgG-aCL – highly positive, 6 – medium positive and 2 – low positive patients. IgM-aCL by ELISA was detected in 13 (37%) of 35 IgA-aCL positive patients: 11 – highly positive, 1 – medium positive and 1 – low positive. IgA-aCL was combined with IgG-aCL in 34 patients and with IgM-aCL in 16 patients in the CMA. IgG-aß2GP1 in ELISA was detected in 32 patients with IgA-aCL (24 –highly positive, 5 – medium positive and 3 – low positive) and in 34 – in CMA. IgM-aß2GP1 was combined with IgA-aß2GP1 with the same frequency in both methods (in 13 patients).IgA-aß2GP1 was detected in 30 (48%) of 63 patients. They were combined with both IgG-aCL and IgG-aß2GP1 in all cases in both methods. IgM-aCL and IgM-aß2GP1 were detected in 14 and 11 of 30 patients with IgA-aß2GP1, respectively. The combination of IgA-aß2GP1 with IgG-aCL by ELISA was in 27 (in most cases highly positive – 20) and with IgM-aCL – in 10 (highly positive - 8). IgG-aß2GP1 was detected in 28 patients with IgA-aß2GP1 (high positive – 21) and in 11 patients with IgM-aß2GP1 (high positive –7).IgG-aß2GP1-D1 was revealed in 48 (76%) patients. It was combined with IgG-aCL – in 38, with IgM-aCL – in 15 patients by the ELISA. The combination of IgG-aß2GP1-D1 by CMA was as follows: with IgG-aCL – in 46, with IgM-aCL – in 17, and with IgA-aCL – in 33 patients. In most cases, IgG-aß2gp1-D1 was combined with highly positive aCL levels. IgG-aß2GP1-D1 positivity was associated with IgG-aß2GP1 positivity in 42 – by ELISA and 47 – by CMA, IgМ-aβ2GP1 – in 13 and 14 patients by ELISA and CMA, respectively, and IgA-aß2GP1 – in 29. Isolated IgG-aß2GP1-D1 positivity was not observed.Conclusion:The frequency of IgA-aCL detection was 56% (35 patients out of 63), IgA-aβ2GP1 – 48% (30 patients out of 63), IgG-aβ2GP1-D1 – 76% (48 patients out of 63). There was not isolated positivity of this “extra” criterial antibodies. The presence of IgA-aCL, IgA-aβ2GP1, IgG-aβ2GP1-D1 was associated with highly positivity of IgG/IgM-aCL and IgG/IgM- aβ2GP1.Disclosure of Interests:None declared

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FRI0224 IDENTIFICATION OF RISK AND PROGNOSTIC FACTORS FOR POLYARTERITIS NODOSA PATIENTS WITH DIGITAL GANGRENE

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.817 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 695.2-695

Author(s):

D. Xu ◽

X. Tian ◽

X. Zeng ◽

F. Zhang ◽

L. Zhao ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Prognostic Factors ◽

Retrospective Study ◽

Polyarteritis Nodosa ◽

Lupus Erythematosus ◽

Disease Duration ◽

Response To Therapy ◽

Smoking History ◽

Systemic Lupus ◽

Poor Outcomes

Background:Polyarteritis nodosa (PAN) is a segmental, necrotizing vascular disease that primarily impacts medium-sized muscle arteries. The estimated annual incidence of PAN is still lacking in China. Digital gangrene is an ischemic manifestation of the limb. However, the causes and the treatment methods vary from case to case, and the outcome is unpredictable. These features emphasize the need to identify measurable variables that accelerate digital gangrene development in PAN patients. However, little effort has been made to identify the clinical and laboratory factors that affect PAN patients with digital gangrene to anticipate their natural history and response to therapy.Objectives:Many patients with polyarteritis nodosa (PAN) complicated with digital gangrene have poor outcomes and related research information is limited. This study was carried out to identify the associated risk and prognostic factors.Methods:We conducted a retrospective study of 148 PAN patients admitted to Peking Union Medical College Hospital (PUMCH) from September 1986 to December 2018. The characteristics, therapeutic regimens, and outcome data for patients with and without gangrene were compared. The Kaplan–Meier method and Cox hazard regression model were used to evaluate the prognostic factors.Results:Forty-seven (31.8%) PAN patients had digital gangrene complications. The average age was 40.4±17.9 years and the average disease duration was 11 (4-27) months. The presence of digital gangrene was correlated with smoking history [odds ratio (OR), 4.27; 95% confidence interval (95% CI), 1.56-11.66] and eosinophil elevation (28.12; 10.30-76.8). Thirty-two (68.1%) gangrene patients received methylprednisolone pulse therapy and all of these patients were treated with cyclophosphamide. Nine patients suffered irreversible organ injury and two died. Disease duration ≥ 24 months and elevated serum C-reactive protein (CRP) were identified as hazardous factors for poor prognosis in patients with gangrene (P=0.003, HR=8.668, 95% CI 2.11, 35.55 andP=0.042, HR=27.062, 95% CI 1.13, 648.57, respectively).Conclusion:Smoking history and eosinophil elevation in PAN patients were more prone to digital gangrene and high serum CRP level predicted poor outcomes. PAN patients with smoking history and elevated eosinophils need to be seriously evaluated by clinicians. Furthermore, the CRP level should be efficiently controlled for good prognosis.References:[1]De Virgilio A, Greco A, Magliulo G, Gallo A, Ruoppolo G, Conte M, et al. Polyarteritis nodosa: A contemporary overview. Autoimmun Rev. 2016;15:564-70.[2]Pagnoux C, Seror R, Henegar C, Mahr A, Cohen P, Le Guern V, et al. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum. 2010;62:616-26.[3]Xu D, You X, Wang Z, Zeng Q, Xu J, Jiang L, et al. Chinese Systemic Lupus Erythematosus Treatment and Research Group Registry VI: Effect of Cigarette Smoking on the Clinical Phenotype of Chinese Patients with Systemic Lupus Erythematosus. PLoS One. 2015;10:e0134451.Acknowledgments:NoDisclosure of Interests:Dong Xu: None declared, Xinping Tian: None declared, Xiaofeng Zeng Consultant of: MSD Pharmaceuticals, Fengchun Zhang: None declared, Lin Zhao: None declared, Shangzhu Zhang: None declared, Jiaxin Zhou: None declared, Jiu-liang Zhao: None declared, Xiaodan Kong: None declared

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Disease Damage Accrual and Low Bone Mineral Density in Female Patients with Systemic Lupus Erythematosus

Biological Research For Nursing ◽

10.1177/10998004211005550 ◽

2021 ◽

pp. 109980042110055

Author(s):

María Correa-Rodríguez ◽

Gabriela Pocovi-Gerardino ◽

José-Luis Callejas-Rubio ◽

Raquel Ríos-Fernández ◽

Blanca Rueda-Medina ◽

...

Keyword(s):

Bone Mineral Density ◽

Systemic Lupus Erythematosus ◽

Lumbar Spine ◽

Bone Mass ◽

Disease Activity ◽

Lupus Erythematosus ◽

Mineral Density ◽

Systemic Lupus ◽

Damage Accrual ◽

Disease Damage

Osteoporosis is a common comorbidity in patients with systemic lupus erythematosus (SLE), but the potential contribution of disease-associated factors to bone status in SLE is not well known because the reported risk factors from different studies differ greatly. We aimed to examine frequency of reduced bone mass in women with SLE, and determine their potential associations with disease activity, damage accrual and SLE-related clinical markers. A cross-sectional study including 121 Caucasian pre-menopausal and postmenopausal women was conducted (mean age 49.2 ± 12.4 years). The SLE Disease Activity Index (SLEDAI-2 K) and the SDI Damage Index were used to assess disease activity and disease-related damage, respectively. Bone mineral density (BMD) of the left femoral neck and lumbar spine (L2–L4) were measured by dual-energy X-ray absorptiometry. Ten patients (8.3%) had osteoporosis, 63 (52.1%) patients had osteopenia and 6.8% of women had history of previous fracture. Patients with low bone mass had a significantly higher mean SDI (1.3 ± 1.2 versus 0.7 ± 1.0 p = 0.003). T-score at lumbar spine was inversely correlated with SDI score (r = -0.222, p = 0.014) and complement C3 level ( r = −0.206, p = .024). SDI scores were significantly different between patients with osteoporosis, osteopenia, and normal BMD after adjusting for covariates ( p = .004). There is a high prevalence of low BMD in Caucasian women with SLE, and this status was associated with higher damage accrual scores, supporting that disease damage may itself be a major contributor to the low BMD. Women with SLE with organ damage require regular bone status monitoring to prevent further musculoskeletal damage.

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AB0421 EFFECT OF BODY WEIGHT ON COMPLEMENT LEVELS IN SYSTEMIC LUPUS ERYTHEMATOSUS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.4097 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1510.3-1510

Author(s):

L. Kondrateva ◽

T. Popkova ◽

E. Nasonov ◽

A. Lila

Keyword(s):

Systemic Lupus Erythematosus ◽

Disease Activity ◽

Lupus Erythematosus ◽

Disease Duration ◽

Normal Weight ◽

World Health ◽

Systemic Lupus ◽

Obesity Status ◽

Health Organization ◽

The Complement System

Background:The complement system is a recognized biomarker for diagnosis or monitoring of disease activity in systemic lupus erythematosus (SLE) patients (pts). But on the other hand, it has been linked to insulin resistance and obesity in general population.Objectives:To find out whether overweight/obesity can modify C3 or C4 levels in SLE pts.Methods:A total of 92 SLE pts (83 women, 9 men, 39 [34;47] years old) were enrolled in the study. Median disease duration was 6[2;14] years, and SLE activity using SLEDAI-2K was 4[2;8]. SLE pts were treated with glucocorticoids (89%), hydroxychloroquine (78%), immunosuppressants (28%), biologics (10%). The overweight/obesity status was determined by World Health Organization criteria in patients with body mass index (BMI) ≥25kg/m2.Results:Overweight/obesity were established in 46% SLE pts. Overweight/obese SLE pts were older than pts with normal BMI (40[36;48] vs 37[31;44] years, р=0,02), and had lower SLEDAI-2K (3[2;6] vs 6[4;8], p<0,01). Lower C3 concentrations were found in 36% overweight/obese pts vs 68% pts with normal weight (р<0,01), decreased C4 levels - in 19% vs 30% pts (p=0,33), median C3 concentrations were 0,98[0,81;1,14] g/l vs 0,84[0,69;0;96] g/l (р<0,01), and C4 levels were 0,15[0,10;0,19] g/l vs 0,12[0,09;0,16] g/l, respectively (p=0,03). C3 and C4 levels negatively correlated with SLEDAI-2K (r=-0,5, p<0,01 for both), the effect was more strongly pronounced in patients with BMI≥25kg/m2 (r=-0,6, p<0,01 for both) than in those with normal weight (r=-0,2, p=0,09 for C3, r=-0,3, p=0,04 for C4).Conclusion:Overweight/obesity status in SLE pts was associated with increased levels of complement proteins, therefore decreased C3 or C4 levels in patients with BMI≥25kg/m2 are more likely related to disease activity and, can potentially induce SLE flares.Disclosure of Interests: :None declared

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Longterm Outcomes and Damage Accrual in Patients with Childhood Systemic Lupus Erythematosus with Psychosis and Severe Cognitive Dysfunction

The Journal of Rheumatology ◽

10.3899/jrheum.121096 ◽

2013 ◽

Vol 40 (4) ◽

pp. 513-519 ◽

Cited By ~ 20

Author(s):

Lily Siok Hoon Lim ◽

Arlette Lefebvre ◽

Susanne Benseler ◽

Earl D. Silverman

Keyword(s):

Systemic Lupus Erythematosus ◽

Disease Activity ◽

Cognitive Dysfunction ◽

Lupus Erythematosus ◽

Clinical Course ◽

Immunosuppressive Treatment ◽

Damage Index ◽

Response To Treatment ◽

Systemic Lupus ◽

Damage Accrual

Objective.(1) To describe the clinical course and response to treatment; and (2) to evaluate and compare damage accrual of distinct phenotypic subgroups of patients with clinically important psychiatric illness of pediatric systemic lupus erythematosus (pSLE).Methods.A single-center cohort study of patients with pSLE followed at a pediatric lupus clinic from 1985 to July 2009. Clinical course and response to treatment were studied. Remission was defined by absence of psychiatric/cognitive symptoms while receiving minimal doses of prednisone. Disease activity and damage were measured using SLE Disease Activity Index and SLE Damage Index.Results.Fifty-three children were included: 40 with psychosis and cognitive dysfunction (PSYC group) and 13 with isolated cognitive dysfunction (COG group). All received immunosuppressive treatment. Eighteen of 32 treated with azathioprine required a change to cyclophosphamide for poor response but none on cyclophosphamide required a change. The median times to remission were 72 weeks (PSYC) and 70 weeks (COG). Eight patients (7 PSYC, 1 COG) experienced flare following response/remission. New damage was noted in 50% of children at a median of 11 months: 57% of PSYC group, 31% of COG group. Persistent cognitive dysfunction was seen in 16% of PSYC patients and 15% of COG patients.Conclusion.Most patients responded to immunosuppressive treatment, although median time to remission was > 1 year. Roughly half the patients acquired a new damage item, most of which did not interfere with functional abilities. Fewer than 20% of patients developed neuropsychiatric damage. Both phenotypes of psychiatric pSLE responded equally well to current treatment.

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

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.5406 ◽

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

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Increased Serum Soluble OX40 in Patients with Systemic Sclerosis

The Journal of Rheumatology ◽

10.3899/jrheum.080120 ◽

2008 ◽

Vol 35 (12) ◽

pp. 2359-2362 ◽

Cited By ~ 41

Author(s):

KAZUHIRO KOMURA ◽

AYUMI YOSHIZAKI ◽

MASANARI KODERA ◽

YOHEI IWATA ◽

FUMIHIDE OGAWA ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Systemic Sclerosis ◽

Lupus Erythematosus ◽

Tumor Necrosis ◽

Disease Duration ◽

Tumor Necrosis Factor Receptor ◽

Healthy Individuals ◽

Systemic Lupus ◽

Clinical Associations ◽

Necrosis Factor

ObjectiveTo determine levels of serum soluble OX40 (also termed CD134, a member of the tumor necrosis factor receptor superfamily) and their clinical associations in patients with systemic sclerosis (SSc).MethodsSerum soluble OX40 levels were examined by ELISA in 53 patients with SSc, 15 patients with systemic lupus erythematosus (SLE), and 32 healthy individuals.ResultsOX40 levels were significantly elevated in SSc patients (125.7 ± 5.7 pg/ml) compared to patients with SLE (80.7 ± 1.7 pg/ml; p < 0.005) and controls (88.2 ± 3.0 pg/ml; p < 0.0001). Elevated OX40 levels were found to be associated with disease duration of less than 2 years (p < 0.05).ConclusionOur results suggest that serum soluble OX40 levels correlate with the early-onset of SSc disease.

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