Which patients with systemic lupus erythematosus in remission can withdraw low dose steroids? Results from a single inception cohort study

Lupus ◽  
2021 ◽  
Vol 30 (6) ◽  
pp. 991-997
Author(s):  
Serena Fasano ◽  
Melania Alessia Coscia ◽  
Luciana Pierro ◽  
Francesco Ciccia
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Low Dose ◽  
Cox Regression ◽  
Systemic Lupus ◽  
Cox Regression Analysis ◽  
History Of ◽  
Risk Of Disease ◽  
Flare Index ◽  
Maintenance Group

Background A progressive tapering until withdrawal of glucocorticoids (GC) is considered one of the main goals of Systemic Lupus Erythematosus (SLE) management. However, which patient may be a candidate for safe GC withdrawal has not been determined yet. This study aimed to evaluate the rate of low-dose GC withdrawal in SLE patients in remission and to identify predictors of flares. Methods Eligible patients were SLE patients in prolonged clinical remission defined by a cSLEDAI = 0 for at least 2 years and on a stable SLE treatment (including daily 5 mg prednisone). Flares were defined by SELENA-SLEDAI Flare Index. Predictors of flares after GC withdrawal were analyzed by Cox regression. Results We selected 56 patients in whom a GC withdrawal was attempted. 98 patients were in the prednisone maintenance group. The proportion of patients experiencing a flare was not significantly lower in the maintenance group than in the withdrawal group (p = 0.81). However, among the withdrawal group, the rate of flares was significantly higher in serologically active clinically quiescent (SACQ) patients (p < 0,0001). At Cox regression analysis, duration of hydroxychloroquine (HCQ) therapy and ≥5 year remission at withdrawal were protective factors, while a SACQ disease and history of lupus nephritis increased the risk of disease flare. Conclusion GC withdrawal is an achievable target in SLE and may be attempted in patients in complete remission.However, it might underline a caution in patients with SACQ disease who may be at greater risk forflare when GCare discontinued. HCQ therapy and durable remission can significantly reduce the risk.

Seroconversion to antinuclear antibody negativity and its association with disease flare in patients with systemic lupus erythematosus

Lupus ◽  
2020 ◽  
Vol 29 (7) ◽  
pp. 697-704 ◽  
Author(s):  
Oh Chan Kwon ◽  
Yong-Gil Kim ◽  
Jung Hwan Park ◽  
Min-Chan Park
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antinuclear Antibody ◽  
Antinuclear Antibodies ◽  
Cox Regression ◽  
British Isles ◽  
Systemic Lupus ◽  
Free Survival ◽  
Cox Regression Analysis ◽  
Kaplan Meier

Objective To evaluate the rate of seroconversion to antinuclear-antibody negativity in patients with systemic lupus erythematosus and its association with subsequent systemic lupus erythematosus flare risk. Methods Medical records of patients with systemic lupus erythematosus with positive antinuclear antibodies (titer ≥1 : 40) at diagnosis and at least one repeat antinuclear antibody test were reviewed. We determined the frequency of seroconversion to antinuclear antibody negativity among these patients and investigated whether seroconversion to antinuclear antibody negativity was associated with subsequent systemic lupus erythematosus flare risk. The seroconversion to antinuclear antibody negativity was defined as a conversion of positive antinuclear antibodies to a titer below the cut-off of 1 : 40. Systemic lupus erythematosus flare was defined as one new British Isles Lupus Assessment Group A or two new British Isles Lupus Assessment Group B domain scores. To estimate hazard ratios and 95% confidence intervals for systemic lupus erythematosus flare according to seroconversion to antinuclear antibody negativity, Cox regression analysis with adjustment for known systemic lupus erythematosus flare risk factors was performed. Kaplan-Meier analysis was used to compare flare-free survival rates between negative converters and non-converters. Results Among the total 175 patients, seroconversion to antinuclear antibody negativity was found in 17 (9.7%) patients in a median 53.5 (range: 25.7–84.0) months. After the last antinuclear antibody tests, 53 systemic lupus erythematosus flare cases were identified during 14.3 (range: 8.2–21.7) months of follow-up. Systemic lupus erythematosus flare risk was significantly lower in patients with negatively seroconverted antinuclear antibodies (adjusted hazard ratio 0.13, 95% confidence interval 0.03–0.58, p = 0.007). Kaplan-Meier analysis showed significantly higher flare-free survival in negative converters than in non-converters ( p = 0.004). Conclusion Seroconversion to antinuclear antibody negativity occurred in 9.7% of patients over 53.5 months and was associated with a lower future systemic lupus erythematosus flare risk.

scholarly journals Clinical and Serological Associations with the Development of Incident Proteinuria in Danish Patients with Systemic Lupus Erythematosus

2018 ◽  
Vol 45 (7) ◽  
pp. 934-941 ◽  
Author(s):  
Nima Tanha ◽  
Renata Baronaite Hansen ◽  
Christoffer Tandrup Nielsen ◽  
Mikkel Faurschou ◽  
Søren Jacobsen
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Cox Regression ◽  
Subsequent Development ◽  
Systemic Lupus ◽  
Vital Status ◽  
Younger Age ◽  
History Of ◽  
Dsdna Antibodies

Objective.In a longitudinal cohort study, we investigated whether clinical and serological manifestations at the time of classification of systemic lupus erythematosus (SLE) were predictive of subsequent development of incident proteinuria as a biomarker of incident lupus nephritis.Methods.Patients fulfilling SLE classification criteria but having no proteinuria prior to or at the time of classification were included. Data on SLE manifestations, vital status, criteria-related autoantibodies, and SLE-associated medications were collected during clinical visits and supplemented by chart review. HR were calculated by Cox regression analyses.Results.Out of 850 patients with SLE, 604 had not developed proteinuria at the time of SLE classification. Of these 604 patients, 184 (30%) developed incident proteinuria following SLE classification. The patients had a median followup of 11 years and 7 months. Younger age and history of psychosis at the time of classification were associated with development of incident proteinuria, just as were lymphopenia (HR 1.49, 95% CI 1.08–2.06), anti-dsDNA (HR 1.38, 95% CI 1.01–1.87), and a high number of autoantibodies (HR 1.26, 95% CI 1.06–1.48).Conclusion.The risk of incident proteinuria after onset of SLE was increased by the presence of lymphopenia, anti-dsDNA antibodies, psychosis, younger age, and a high number of autoantibodies at onset.

Prognostic Indicators of Hospitalized Patients with Systemic Lupus Erythematosus: A Large Retrospective Multicenter Study in China

2011 ◽  
Vol 38 (7) ◽  
pp. 1289-1295 ◽  
Author(s):  
XUEBING FENG ◽  
YAOHONG ZOU ◽  
WENYOU PAN ◽  
XIANGDANG WANG ◽  
MIN WU ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Cox Regression ◽  
Disease Onset ◽  
Antimalarial Drugs ◽  
Hospitalized Patients ◽  
Systemic Lupus ◽  
Female Ratio ◽  
Cox Regression Analysis ◽  
Factors Associated

Objective.To investigate the mortality of hospitalized patients with systemic lupus erythematosus (SLE) and determine the influential factors associated with poor prognosis.Methods.Medical records of 1956 SLE inpatients from 15 hospitals during the period January 1, 1999, to December 31, 2009, were reviewed. All patients were followed up in January 2010. Potential factors associated with mortality were analyzed, comparing patients who were living with those who were deceased. The independency of those factors significantly related to death was determined by Cox regression analysis.Results.Male to female ratio was 1:15 in this cohort; median age at disease onset was 30 years. Hematologic (70.0%), mucocutaneous (68.2%), musculoskeletal (57.9%), and renal (48.7%) involvements were most often seen in these patients at time of admission. The overall mortality was 8.5% (n = 166), with infection (25.9%), renal failure (19.3%), and neuropsychiatric lupus (18.7%) the leading 3 causes of death. Independent predictors for mortality in this cohort of SLE patients were neuropsychiatric involvement [hazard ratio (HR) 2.19], anemia (HR 1.69), SLEDAI score > 8 at discharge (HR 1.64), increased serum creatinine (HR 1.57), low serum albumin (HR 1.56), cardiopulmonary involvement (HR 1.55), and patient untreated before admission (HR 1.48), whereas the use of antimalarial drugs (HR 0.62) and positive anti-Sm antibody (HR 0.60) were shown to be protective factors.Conclusion.SLE patients with delayed treatment and refractory disease have poorer prognosis. A high incidence of death would be expected if they have neuropsychiatric involvement, anemia, azotemia, or cardiopulmonary involvement. Combination therapy with antimalarial drugs may provide some benefit to patients with SLE.

Longterm Hydroxychloroquine Therapy and Low-dose Aspirin May Have an Additive Effectiveness in the Primary Prevention of Cardiovascular Events in Patients with Systemic Lupus Erythematosus

2017 ◽  
Vol 44 (7) ◽  
pp. 1032-1038 ◽  
Author(s):  
Serena Fasano ◽  
Luciana Pierro ◽  
Ilenia Pantano ◽  
Michele Iudici ◽  
Gabriele Valentini
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Low Dose ◽  
Thrombotic Event ◽  
Systemic Lupus ◽  
Cox Regression Analysis ◽  
Dose Aspirin ◽  
Kaplan Meier ◽  
Increased Risk ◽  
Low Dose Aspirin

Objective.Systemic lupus erythematosus (SLE) is associated with an increased risk of cardiovascular disease (CVD). Thromboprophylaxis with low-dose aspirin (ASA) and hydroxychloroquine (HCQ) seems promising in SLE. We investigated the effects of HCQ cumulative dosages (c-HCQ) and the possible synergistic efficacy of ASA and HCQ in preventing a first CV event (CVE) in patients with SLE.Methods.Patients consecutively admitted to our center who, at admission, satisfied the 1997 American College of Rheumatology and/or 2012 Systemic Lupus Collaborating Clinics classification criteria for SLE, and had not experienced any CVE, were enrolled. The occurrence of a thrombotic event, use of ASA, and c-HCQ were recorded. Kaplan-Meier analysis was performed to determine the c-HCQ associated with a lower incidence of CVE. Cox regression analysis served to identify factors associated with a first CVE.Results.For the study, 189 patients with SLE were enrolled and monitored for 13 years (median). Ten CVE occurred during followup. At Kaplan-Meier analysis, the CVE-free rate was higher in ASA-treated patients administered a c-HCQ > 600 g (standard HCQ dose for at least 5 yrs) than in patients receiving ASA alone, or with a c-HCQ dose < 600 g (log-rank test chi-square = 4.01, p = 0.04). Multivariate analysis showed that antimalarials plus ASA protected against thrombosis (HR 0.041 and HR 0.047, respectively), while antiphospholipid antibodies (HR 17.965) and hypertension (HR 18.054) increased the risk of a first CVE.Conclusion.Our results suggest that prolonged use of HCQ plus ASA is thromboprotective in SLE and provides additional evidence for its continued use in patients with SLE.

scholarly journals Ultrasensitive serum interferon-α quantification during SLE remission identifies patients at risk for relapse

2019 ◽  
Vol 78 (12) ◽  
pp. 1669-1676 ◽  
Author(s):  
Alexis Mathian ◽  
Suzanne Mouries-Martin ◽  
Karim Dorgham ◽  
Hervé Devilliers ◽  
Hans Yssel ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Single Molecule ◽  
Lupus Erythematosus ◽  
Cox Regression ◽  
Activity Index ◽  
Serum Levels ◽  
Interferon Α ◽  
Systemic Lupus ◽  
Cox Regression Analysis ◽  
Time To Relapse

ObjectivesMaintenance of remission has become central in the management of systemic lupus erythematosus (SLE). The importance of interferon-alpha (IFN-α) in the pathogenesis of SLE notwithstanding, its expression in remission has been poorly studied as yet. To study its expression in remission and its prognostic value in the prediction of a disease relapse, serum IFN-α levels were determined using an ultrasensitive single-molecule array digital immunoassay which enables the measurement of cytokines at physiological concentrations.MethodsA total of 254 SLE patients in remission, according to the Definition of Remission in SLE classification, were included in the study. Serum IFN-α concentrations were determined at baseline and patients were followed up for 1 year. Lupus flares were defined according to the Safety of Estrogens in Lupus Erythematosus: National Assessment version of the Systemic Lupus Erythematosus Disease Activity Index Flare Index, whereas the Kaplan-Meier analysis and Cox regression analysis were used to estimate the time to relapse and to identify baseline factors associated with time to relapse, respectively.ResultsOf all patients in remission, 26% displayed abnormally high IFN-α serum levels that were associated with the presence of antibodies specific for ribonucleoprotein (RNP), double stranded (ds)DNA and Ro/SSA60, as well as young age. Importantly, elevated-baseline IFN-α serum levels and remission duration were associated in an independent fashion, with shorter time to relapse, while low serum levels of complement component 3 and anti-dsDNA Abs were not.ConclusionDirect serum IFN-α assessment with highly sensitive digital immunoassay permits clinicians to identify a subgroup of SLE patients, clinically in remission, but at higher risk of relapse.

Hereditary C1 inhibitor deficiency associated with systemic lupus erythematosus

Lupus ◽  
2020 ◽  
Vol 29 (11) ◽  
pp. 1456-1460
Author(s):  
Anuj Shukla ◽  
Priyanka Gaur
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Mycophenolate Mofetil ◽  
Lupus Erythematosus ◽  
Low Dose ◽  
C1 Inhibitor ◽  
Systemic Lupus ◽  
C1 Inhibitor Deficiency ◽  
Life Threatening ◽  
History Of ◽  
Control Disease

Here, we report a family with two children (the elder son and younger daughter) diagnosed with juvenile-onset systemic lupus erythematosus (SLE) and the father diagnosed with hereditary angioedema. Serum C1 inhibitor (C1-INH) levels were low, and clinical exome next-generation sequencing detected a frameshift mutation in the SERPING-1 gene in all three patients. The mother had neither of the clinical phenotypes. The son had cutaneous symptoms, fever and polyarthralgia, along with lupus nephritis, and thus required rituximab therapy as well as mycophenolate mofetil and low-dose steroids to control disease activity. The daughter had a milder disease, with cutaneous manifestation, fever and polyarthralgia, and which was controlled with mycophenolate mofetil, hydroxychloroquine and low-dose steroids. Both children had never experienced angioedema. The father had a long history of self-limiting, non-life-threatening irregular episodes of subcutaneous angioedema and abdomen pain. He was not on any regular medication for these symptoms. We searched the literature for evidence of hereditary C1-INH deficiency associated with monogenic SLE or SLE-like-phenotype.

scholarly journals lncRNA MALAT-1 Predicts Prognosis of Patients with Systemic Lupus Erythematosus

2021 ◽  
Author(s):  
Aihong Zhou ◽  
Kunyun Mao ◽  
Guoqiang Liu ◽  
Jun Li ◽  
Xia Lin
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Regression Analysis ◽  
Lupus Erythematosus ◽  
Cox Regression ◽  
Healthy Controls ◽  
Confounding Factors ◽  
Systemic Lupus ◽  
Cox Regression Analysis ◽  
Expression Levels ◽  
Risk Of Mortality

Abstract Objective To evaluate the association of MALAT-1 and the prognosis of patients with systemic lupus erythematosus (SLE). Materials and Methods A total of 87 SLE patients and 50 healthy controls were recruited. The expression levels of MALAT-1 were measured by quantitative PCR at baseline. Multivariate Cox regression analysis was performed to assess the association of MALAT-1 levels with survival after adjusting for potential confounding factors. Results The MALAT-1 levels in patients with SLE were significantly higher compared with healthy controls (p<0.01). The 10-year survival of patients with a higher MALAT-1 level was significantly lower than that of patients with a lower MALAT-1 level (HR=5.54; 95% CI: 1.49–20.55; p=0.01). Multivariate Cox regression indicated that the MALAT-1 level was significantly associated with a risk of mortality (HR=10.02, 95% CI: 1.14–88.14, p=0.04). Conclusion The expression levels of MALAT-1 may be a promising biomarker for evaluating the prognosis of SLE patients.

scholarly journals Predictors of renal damage in systemic lupus erythematous patients: data from a multiethnic, multinational Latin American lupus cohort (GLADEL)

RMD Open ◽  
2020 ◽  
Vol 6 (3) ◽  
pp. e001299
Author(s):  
Cristina Reátegui-Sokolova ◽  
Manuel F Ugarte-Gil ◽  
Guillermina B Harvey ◽  
Daniel Wojdyla ◽  
Guillermo J Pons-Estel ◽  
...  
Keyword(s):  
Risk Factors ◽  
Systemic Lupus Erythematosus ◽  
Latin American ◽  
Lupus Erythematosus ◽  
Renal Damage ◽  
Cox Regression ◽  
Damage Index ◽  
Early Response ◽  
Male Gender ◽  
Systemic Lupus

AimA decrease in proteinuria has been considered protective from renal damage in lupus nephritis (LN), but a cut-off point has yet to be established. The aim of this study was to identify the predictors of renal damage in patients with LN and to determine the best cut-off point for a decrease in proteinuria.MethodsWe included patients with LN defined clinically or histologically. Possible predictors of renal damage at the time of LN diagnosis were examined: proteinuria, low complement, anti-double-stranded DNA antibodies, red cell casts, creatinine level, hypertension, renal activity (assessed by the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)), prednisone dose, immunosuppressive drugs and antimalarial use. Sociodemographic variables were included at baseline. Proteinuria was assessed at baseline and at 12 months, to determine if early response (proteinuria <0.8 g/day within 12 months since LN diagnosis) is protective of renal damage occurrence. Renal damage was defined as an increase of one or more points in the renal domain of The Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index (SDI). Cox regression models using a backward selection method were performed.ResultsFive hundred and two patients with systemic lupus erythematosus patients were included; 120 patients (23.9%) accrued renal damage during their follow-up. Early response to treatment (HR=0.58), antimalarial use (HR=0.54) and a high SES (HR=0.25) were protective of renal damage occurrence, whereas male gender (HR=1.83), hypertension (HR=1.86) and the renal component of the SLEDAI (HR=2.02) were risk factors for its occurrence.ConclusionsEarly response, antimalarial use and high SES were protective of renal damage, while male gender, hypertension and higher renal activity were risk factors for its occurrence in patients with LN.

“Protenuria in SLE: Is it always lupus?”

Lupus ◽  
2021 ◽  
pp. 096120332098345
Author(s):  
Alessandra Ida Celia ◽  
Roberta Priori ◽  
Bruna Cerbelli ◽  
Francesca Diomedi-Camassei ◽  
Vincenzo Leuzzi ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Fabry Disease ◽  
Histological Examination ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Kidney Involvement ◽  
History Of ◽  
Genetic Assay

Proteinuria is one of the most typical manifestations of kidney involvement in Systemic Lupus Erythematosus (SLE). We report the case of a 23-year-old woman with a 6-year-long history of SLE presenting with proteinuria after a three-year remission on hydroxychloroquine. Kidney histological examination showed alterations inconsistent with lupus nephritis and suggestive of hydroxychloroquine toxicity or Fabry disease. The latter was confirmed by genetic assay.

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