Publications by authors named "Guillermo Ruiz-Irastorza"

126 Publications

Eurolupus cyclophosphamide plus repeated pulses of methyl-prednisolone for the induction therapy of class III, IV and V lupus nephritis.

Autoimmun Rev 2021 Jul 15;20(10):102898. Epub 2021 Jul 15.

UMR CNRS5164, ImmunoconcEpT, Bordeaux University, Bordeaux, France; Internal Medicine Department, Haut-Lévêque Hospital, Bordeaux, France; ACRONIM University Hospital Federation, Bordeaux, France.

Objective: To study whether adding repeated 125 mg methyl-prednisolone pulses (MP) to Eurolupus fortnightly intravenous cyclophosphamide (CYC) improves remission of lupus nephritis (LN) compared with recommended schedules.

Methods: Observational comparative study of patients with biopsy-confirmed class III, IV or V LN: 30 in the mycophenolate (MMF) group, 25 in the CYC group and 38 in the CYC-MP group. The main efficacy outcome was complete response at 12 months.

Results: Patients in the CYC-MP group received lower doses of prednisone within 6 months (mean 8.5 mg/d, vs. 15 mg/d in the MMF group vs. 24 mg/d in the CYC group, respectively). The complete response rates at 12 months were: CYC-MP 86%; CYC 56%; MMF 47% (p = 0.002) at Pr/Cr <0.5; CYC-MP 86%; CYC 65%; MMF 63% (p = 0.07) at Pr/Cr ≤0.7. The cumulative 12-month response rates for the CYC-MP, CYC and MMF groups were, respectively, 0.90, 0.58 and 0.63 (p = 0.004). In the adjusted Cox model, patients receiving CYC-MP were more likely to achieve complete response at Pr/Cr <0.5 than those in the MMF (HR vs. CYC-MP 0.33, 95%CI 0.16-0.65) and the CYC groups (HR vs. CYC-MP 0.47, 95%CI 0.21-1.04). Glucocorticoid-related toxicity was seen in 2.6% of the CYC-MP group, 24% of the CYC group and 20% of the MMF group (p = 0.029).

Conclusion: The addition MP of 125 mg to each fortnightly dose of 500 mg of CYC improves response rates and reduces the need for oral glucocorticoids in patients with class III, IV and V LN.
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http://dx.doi.org/10.1016/j.autrev.2021.102898DOI Listing
July 2021

Neuropsychiatric Events in Systemic Lupus Erythematosus.

Arthritis Rheumatol 2021 May 27. Epub 2021 May 27.

Copenhagen Lupus and Vasculitis Clinic, 4242, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.

Objectives: To determine predictors for change in neuropsychiatric (NP) event status in a large, prospective, international, inception cohort of SLE patients METHODS: Upon enrollment and annually thereafter, NP events attributed to SLE and non-SLE causes and physician determined resolution were documented. Factors potentially associated with onset and resolution of NP events were determined by time-to-event analysis using a multistate modelling structure.

Results: NP events occurred in 955/1,827 (52.3%) patients and 592/1910 (31.0%) unique events were attributed to SLE. For SLE NP events multivariate analysis revealed positive associations with male sex, concurrent non-SLE NP events excluding headache, active SLE and corticosteroids. There was a negative association with Asian race/ethnicity, post-secondary education, and immunosuppressive or anti-malarial drugs. For non-SLE NP events, excluding headache, there was a positive association with concurrent SLE NP events and negative associations with African and Asian race/ethnicity. NP events attributed to SLE had a higher resolution rate than non-SLE NP events, with the exception of headache that had comparable resolution rates. For SLE NP events, multivariate analysis revealed resolution was more common with Asian race/ethnicity and for central/focal NP events. For non-SLE NP events resolution was more common with African race/ethnicity and less common with older age at SLE diagnosis.

Conclusions: In a large and long-term study of the occurrence and resolution of NP events in SLE we identified subgroups with better and worse prognosis. The course of NP events differs greatly depending on their nature and attribution.
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http://dx.doi.org/10.1002/art.41876DOI Listing
May 2021

What are the topics you care about making trials in lupus more effective? Results of an Open Space meeting of international lupus experts.

Lupus Sci Med 2021 05;8(1)

Policlinic and Hiller Research Unit, Heinrich Heine University Düsseldorf, Dusseldorf, Nordrhein-Westfalen, Germany.

Despite promising candidates for new therapeutic options in the treatment of systemic lupus erythematosus (SLE), many clinical trials have failed in the past few years. The disappointing results have been at least partly be attributed to trial designs. With the aim of stimulating new developments in SLE trial design, an international open space meeting was held on occasion of the European Lupus Meeting 2018 in Duesseldorf, Germany about 'What are the topics you care about for making trials in lupus more effective?'. The Open Space is a participant-driven technology, where the discussion topics and schedule are selected during the meeting by all participants and discussion rounds are led by the people attending encouraging active contributions. Eleven topics were selected for further discussion, of which 6 were voted to be more intensively discussed in two consecutive rounds. Major topics were the optimal handling of glucocorticoids in clinical trials, the improvement of outcome measures, reducing or controlling the placebo response and the identification of biomarkers and stratification parameters. Further, the importance of local and international networks was emphasised. By networking, collaborations are facilitated, patient recruitment is more efficient and treatment can be harmonised thus lead to more successful SLE trials. Further discussions are needed to substantiate the results and develop new trial designs.
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http://dx.doi.org/10.1136/lupus-2021-000506DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8141446PMC
May 2021

PREDNISONE IN SYSTEMIC LUPUS ERYTHEMATOSUS: TAPER QUICKLY, WITHDRAW SLOWLY.

Rheumatology (Oxford) 2021 Apr 11. Epub 2021 Apr 11.

Autoimmune Diseases Research Unit, Department of Internal Medicine, Biocruces Bizkaia Health Research Institute, Hospital Universitario Cruces, University of The Basque Country, Bizkaia, Spain, The Basque Country.

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http://dx.doi.org/10.1093/rheumatology/keab347DOI Listing
April 2021

ANTIPHOSPHOLIPID ANTIBODIES DO NOT PREDICT DAMAGE IN SLE PATIENTS IN THE XXI CENTURY. AN OBSERVATIONAL STUDY FROM THE LUPUS-CRUCES COHORT.

Rheumatology (Oxford) 2021 Mar 26. Epub 2021 Mar 26.

Autoimmune Diseases Research Unit, Department of Internal Medicine, Biocruces Bizkaia Health Research Institute, Hospital Universitario Cruces, Bizkaia, The Basque Country, Spain.

Objective: To compare the influence of antiphospholipid antibodies (aPL) on global and cardiovascular damage in patients with systemic lupus erythematosus (SLE) diagnosed before and after year 2000.

Methods: 286 patients from the Lupus-Cruces cohort with a minimum follow-up of 5 years, divided into two sub-cohorts according to the date of diagnosis, before 2000 (<2000) and from 2000 on (≥2000). We compared the mean SDI score and global and cardiovascular damage-free survival rates in the presence/absence of aPL in both sub-cohorts. Variables potentially modulating damage among aPL-positive patients were analysed.

Results: The sub-cohorts were comparable for demographic and lupus-related variables except for treatment variables: the ≥2000 sub-cohort received lower doses of prednisone and more hydroxychloroquine, low-dose aspirin, statins, immunosuppressive agents and Vitamin D. aPL-positive patients in the <2000, but not in the ≥2000 sub-cohort, accrued more damage compared with aPL-negative. In the <2000 sub-cohort, the adjusted HRs for global and cardiovascular damage in aPL-positive vs. aPL-negative patients were 1.98 (95% CI 1.24-3.14) and 9.3 (95% CI 3.24-26.92), respectively. No differences in damage were seen between aPL-positive and aPL-negative patients in the ≥2000 sub-cohort. Hypertension (HR 4.64, 95%CI 1.33-16.19), lupus anticoagulant (HR 3.85, 95%CI 1.1-13.41) and the number of months on hydroxychloroquine (HR 0.97, 95%CI 0.95-0.99) were independent predictors of vascular damage in the combined analysis of all aPL-positive patients.

Conclusion: The effects of aPL on damage accrual in SLE patients have been reduced over the last years. The widespread use of hydroxychloroquine and a better thromboprophylaxis are likely causing this change.
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http://dx.doi.org/10.1093/rheumatology/keab307DOI Listing
March 2021

European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) SLE classification criteria item performance.

Ann Rheum Dis 2021 Feb 10. Epub 2021 Feb 10.

Department of Medicine, Division of Rheumatology, University of California at San Francisco and the VA Medical Center, San Francisco, California, USA.

Background/objectives: The European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) 2019 classification criteria for systemic lupus erythematosus system showed high specificity, while attaining also high sensitivity. We hereby analysed the performance of the individual criteria items and their contribution to the overall performance of the criteria.

Methods: We combined the EULAR/ACR derivation and validation cohorts for a total of 1197 systemic lupus erythematosus (SLE) and n=1074 non-SLE patients with a variety of conditions mimicking SLE, such as other autoimmune diseases, and calculated the sensitivity and specificity for antinuclear antibodies (ANA) and the 23 specific criteria items. We also tested performance omitting the EULAR/ACR criteria attribution rule, which defines that items are only counted if not more likely explained by a cause other than SLE.

Results: Positive ANA, the new entry criterion, was 99.5% sensitive, but only 19.4% specific, against a non-SLE population that included other inflammatory rheumatic, infectious, malignant and metabolic diseases. The specific criteria items were highly variable in sensitivity (from 0.42% for delirium and 1.84% for psychosis to 75.6% for antibodies to double-stranded DNA), but their specificity was uniformly high, with low C3 or C4 (83.0%) and leucopenia <4.000/mm³ (83.8%) at the lowest end. Unexplained fever was 95.3% specific in this cohort. Applying the attribution rule improved specificity, particularly for joint involvement.

Conclusions: Changing the position of the highly sensitive, non-specific ANA to an entry criterion and the attribution rule resulted in a specificity of >80% for all items, explaining the higher overall specificity of the criteria set.
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http://dx.doi.org/10.1136/annrheumdis-2020-219373DOI Listing
February 2021

Lower vitamin D is associated with metabolic syndrome and insulin resistance in systemic lupus: data from an international inception cohort.

Rheumatology (Oxford) 2021 Feb 8. Epub 2021 Feb 8.

Department of Rheumatology, Kantonsspital Schaffhausen, Schaffhausen, Schaffhausen, CH.

Objectives: Vitamin D (25(OH)D) deficiency and metabolic syndrome (MetS) may both contribute to increased cardiovascular risk in systemic lupus erythematosus (SLE). We aimed to examine the association of demographic factors, SLE phenotype, therapy and vitamin D levels with MetS and insulin resistance.

Methods: The Systemic Lupus International Collaborating Clinics (SLICC) enrolled patients recently diagnosed with SLE (<15 months) from 33 centres across 11 countries from 2000. Clinical, laboratory and therapeutic data were collected. Vitamin D level was defined according to tertiles based on distribution across this cohort, which were set at T1 (10-36 nmol/l), T2 (37-60 nmol/l) and T3 (61-174 nmol/l). MetS was defined according to the 2009 consensus statement from the International Diabetes Federation. Insulin resistance was determined using the HOMA-IR model. Linear and logistic regressions were used to assess the association of variables with vitamin D levels.

Results: Of the 1847 patients, 1163 (63%) had vitamin D measured and 398 (34.2%) subjects were in the lowest 25(OH)D tertile. MetS was present in 286 of 860 (33%) patients whose status could be determined. Patients with lower 25(OH)D were more likely to have MetS and higher HOMA-IR. The MetS components, hypertension, hypertriglyceridemia and decreased HDL were all significantly associated with lower 25(OH)D. Increased average glucocorticoid exposure was associated with higher insulin resistance.

Conclusions: MetS and insulin resistance are associated with lower vitamin D in patients with SLE. Further studies could determine whether vitamin D repletion confers better control of these cardiovascular risk factors and improve long-term outcomes in SLE.
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http://dx.doi.org/10.1093/rheumatology/keab090DOI Listing
February 2021

Transplantation-mediated alloimmune thrombocytopenia successfully treated by retransplantation.

Lupus 2021 Apr 6;30(4):669-673. Epub 2021 Jan 6.

General and Digestive Surgery Department, Hospital Universitario Cruces, Bizkaia, Spain.

Introduction: Transplantation-mediated alloimmune thrombocytopenia (TMAT) is a rare complication affecting the recipient of an organ from a donor with immune thrombocytopenia (ITP).

Methods: We present a case of TMAT following liver transplantation successfully treated by retransplantation, along with a review of previously published cases.Clinical presentation: The liver donor had lupus and ITP and died from an intracranial hemorrhage. The recipient's platelet count fell to x10/L on postoperative day 2. Due to the lack of response to medical treatment, emergency retransplantation was undertaken with a steady recovery of the platelet count within a few days.

Discussion: Six additional cases of transplantation-mediated alloimmune thrombocytopenia after liver transplantation have been reported. In all cases, severe thrombocytopenia ensued within 3 days after liver transplantation. Four patients suffered hemorrhagic complications. Three patients died. Early retransplantation was needed in three out of four patients receiving a graft from a donor with ITP and splenectomy. All recovered shortly after the new graft was in place.

Conclusion: Severe refractory transplantation-mediated alloimmune thrombocytopenia can develop in liver recipients from donors with ITP, especially those with previous splenectomy. Early retransplantation should be considered if there is no rapid response to medical therapy.
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http://dx.doi.org/10.1177/0961203320983450DOI Listing
April 2021

Treating systemic lupus erythematosus in the 21st century: new drugs and new perspectives on old drugs.

Rheumatology (Oxford) 2020 12;59(Suppl5):v69-v81

Rheumatology, Clinical Immunology and Allergy, University of Crete Medical School and University Hospital of Iraklio, Iraklio, Greece.

Besides treating acute flares, the management of SLE should aim at preventing organ damage accrual and drug-associated harms, improving health-related quality of life and prolonging survival. At present, therapy is based on combinations of antimalarials (mainly HCQ), considered the backbone of SLE treatment, glucocorticoids and immunosuppressive drugs. However, these regimens are not universally effective and a substantial degree of damage can be caused by exposure to glucocorticoids. In this review we provide a critical appraisal of the efficacy and safety of available treatments as well as a brief discussion of potentially novel compounds in patients with SLE. We emphasize the use of methylprednisolone pulses for moderate-severe flares, followed by low-moderate doses of oral prednisone with quick tapering to maintenance doses of ≤5 mg/day, as well as the prompt institution of immunosuppressive drugs in the setting of severe disease but also as steroid-sparing agents. Indications for the use of biologic agents, namely belimumab and rituximab, in refractory or organ-threatening disease are also presented. We conclude by proposing evidence- and experience-based treatment strategies tailored to the clinical scenario and prevailing organ involvement that can aid clinicians in managing this complex disease.
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http://dx.doi.org/10.1093/rheumatology/keaa403DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7719039PMC
December 2020

Hydroxychloroquine Retinal Toxicity and Its Association with Dosage.

J Rheumatol 2021 01 1;48(1):150. Epub 2020 Dec 1.

Hospital Universitario Cruces, Autoimmune Diseases Research Unit.

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http://dx.doi.org/10.3899/jrheum.200843DOI Listing
January 2021

Prediction of hospitalizations in systemic lupus erythematosus using the Systemic Lupus International Collaborating Clinics Frailty Index (SLICC-FI).

Arthritis Care Res (Hoboken) 2020 Nov 5. Epub 2020 Nov 5.

Emory University School of Medicine, Division of Rheumatology, Atlanta, Georgia, USA.

Objective: The Systemic Lupus International Collaborating Clinics (SLICC) frailty index (FI) predicts mortality and damage accrual in SLE, but its association with hospitalizations has not been described. We estimated the association of baseline SLICC-FI values with future hospitalizations in the SLICC inception cohort.

Methods: Baseline SLICC-FI scores were calculated. The number and duration of inpatient hospitalizations during follow-up were recorded. Negative binomial regression was used to estimate the association between baseline SLICC-FI values and the rate of hospitalizations per patient-year of follow-up. Linear regression was used to estimate the association of baseline SLICC-FI scores with the proportion of follow-up time spent in hospital. Multivariable models were adjusted for relevant baseline characteristics.

Results: The 1549 SLE patients eligible for this analysis were mostly female (88.7%) with mean (SD) age 35.7 (13.3) years and median (IQR) disease duration 1.2 (0.9-1.5) years at baseline. Mean (SD) baseline SLICC-FI was 0.17 (0.08). During mean (SD) follow-up of 7.2 (3.7) years, 614 patients (39.6%) experienced 1570 hospitalizations. Higher baseline SLICC-FI values (per 0.05 increment) were associated with more frequent hospitalizations during follow-up (Incidence Rate Ratio 1.21; 95%CI 1.13-1.30), adjusting for baseline age, sex, corticosteroid use, immunosuppressive use, ethnicity/location, SLE disease activity index 2000 (SLEDAI-2K), SLICC/ACR damage index (SDI), and disease duration. Among patients with ≥1 hospitalization, higher baseline SLICC-FI values predicted a greater proportion of follow-up time spent hospitalized (Relative Rate 1.09; 95%CI 1.02-1.16).

Conclusion: The SLICC-FI predicts future hospitalizations among incident SLE patients, further supporting the SLICC-FI as a valid health measure in SLE.
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http://dx.doi.org/10.1002/acr.24504DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8096857PMC
November 2020

Second week methyl-prednisolone pulses improve prognosis in patients with severe coronavirus disease 2019 pneumonia: An observational comparative study using routine care data.

PLoS One 2020 22;15(9):e0239401. Epub 2020 Sep 22.

Clinical Epidemiology Unit, Hospital Universitario Cruces, Barakaldo, Bizkaia, Spain.

Objective: To analyze the effects of a short course of methyl-prednisolone pulses (MP) during the second week of disease (week-2) in patients with severe coronavirus disease 2019 (COVID-19) pneumonia.

Methods: Comparative observational study using data collected from routine care at Hospital Universitario Cruces, Barakaldo, Bizkaia, Spain in patients with COVID-19 pneumonia. We compared patients who received week-2-MP (125-250 mg/d x3) with those who did not, with the end-points time to death and time to death or endotracheal intubation.

Results: We included 242 patients with COVID-19 pneumonia and elevated inflammatory markers at admission. Sixty-one patients (25%) received week-2-MP. Twenty-two patients (9%) died and 31 (12.8%) suffered death or intubation. The adjusted HRs for death and death or intubation for patients in the week-2-MP group were 0.35 (95%CI 0.11 to 1.06, p = 0.064) and 0.33 (95%CI 0.13 to 0.84, p = 0.020), respectively. These differences were specifically seen in the subcohort of patients with a SpO2/FiO2 at day 7 lower than 353 (adjusted HR 0.31, 95% CI 0.08 to 1.12, p = 0.073 and HR 0.34, 95%CI 0.12 to 0.94, p = 0.038, respectively) but not in patients with higher SpO2/FiO2. Patients receiving out-of-week-2-MP, non-pulse glucocorticoids or no glucocorticoids had an increased adjusted risk for both outcomes compared with week-2-MP group: HR 5.04 (95% CI 0.91-27.86), HR 10.09 (95% CI 2.14-47.50), HR 4.14 (95% CI 0.81-21.23), respectively, for death; HR 7.38 (95% CI 1.86-29.29), HR 13.71 (95% CI 3.76-50.07), HR 3.58 (95% CI 0.89-14.32), respectively, for death or intubation. These differences were significant only in the subgroup with low SpO2/FiO2.

Conclusions: Week-2-MP are effective in improving the prognosis of patients with COVID-19 pneumonia with features of inflammatory activity and respiratory deterioration entering the second week of disease. The recognition of this high-risk population should prompt early use of MP at this point.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0239401PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7508405PMC
October 2020

Update on antimalarials and systemic lupus erythematosus.

Curr Opin Rheumatol 2020 11;32(6):572-582

Autoimmune Diseases Research Unit, Department of Internal Medicine, Biocruces Bizkaia Health Research Institute, Hospital Universitario Cruces, University of The Basque Country, Bizkaia, Spain.

Purpose Of Review: The purpose of this review is highlighting the most recent evidence on the clinical efficacy and toxicity of antimalarials in systemic lupus erythematosus (SLE).

Recent Findings: New data confirm the effects of antimalarials in preventing SLE activity, damage and infections and in decreasing mortality. An important reduction in use of health resources is related to continued antimalarial use. Hydroxychloroquine (HCQ) may prevent preeclampsia in pregnant women with SLE. HCQ ocular toxicity is infrequent and could be associated with blood levels. Gastrointestinal and skin toxicity are underrecognized and could influence adherence. Prolongation of QT interval is extremely unusual with HCQ. Doses of HCQ of 200 mg/day seem to offer a good efficacy/toxicity balance. HCQ protection against herpes zoster and Pneumocystis jirovecii infection has been shown. On the contrary, HCQ prescription by doctors and adherence by patients are both under recommended standards. The recent coronavirus disease 2019 pandemic has resulted in a significant shortage of HCQ in many countries with possible consequences in the correct treatment of lupus patients.

Summary: Recent evidence reinforces the central role of HCQ in SLE therapy. The reduction in activity, damage accrual and mortality is consistent across studies, countries and ethnical groups. On the contrary, and despite the well established beneficial effects of prolonged regular HCQ therapy, many SLE patients do never take this drug or it is eventually stopped in the setting of severe flares, pregnancy or presumed toxicity. Every effort must be made to assure the correct prescription of HCQ and not to withdraw the drug unless unequivocal signs of toxicity are present.
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http://dx.doi.org/10.1097/BOR.0000000000000743DOI Listing
November 2020

Glucocorticoids in . Ten Questions and Some Issues.

J Clin Med 2020 Aug 21;9(9). Epub 2020 Aug 21.

Autoimmune Diseases Research Unit, BioCruces Bizkaia Health Research Institute, Cruces Univeristy Hospital, 48903 Bizkaia, Spain.

Since the discovery of glucocorticoids (GCs), their important anti-inflammatory effect, rapid mechanism of action, low cost, and accessibility have made them one of the mainstays of treatment for (SLE). Although their use has allowed controlling the disease and reducing acute mortality in severe conditions, the implementation of a scheme based on high doses for long periods has inevitably been accompanied by an increase in adverse effects and infections, including long-term damage. The objective of this review is to answer some important questions that may arise from its use in daily clinical practice, and to propose a paradigm based on the use of methylprednisolone pulses followed by medium-low doses and a rapid decrease of prednisone.
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http://dx.doi.org/10.3390/jcm9092709DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7563630PMC
August 2020

Performance of the 2019 EULAR/ACR classification criteria for systemic lupus erythematosus in early disease, across sexes and ethnicities.

Ann Rheum Dis 2020 10 14;79(10):1333-1339. Epub 2020 Aug 14.

Rheumatology, Azienda Ospedaliero Universitaria Pisana, Pisa, Toscana, Italy.

Objectives: The European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) 2019 Classification Criteria for systemic lupus erythematosus (SLE) have been validated with high sensitivity and specificity. We evaluated the performance of the new criteria with regard to disease duration, sex and race/ethnicity, and compared its performance against the Systemic Lupus International Collaborating Clinics (SLICC) 2012 and ACR 1982/1997 criteria.

Methods: Twenty-one SLE centres from 16 countries submitted SLE cases and mimicking controls to form the validation cohort. The sensitivity and specificity of the EULAR/ACR 2019, SLICC 2012 and ACR 1982/1997 criteria were evaluated.

Results: The cohort consisted of female (n=1098), male (n=172), Asian (n=118), black (n=68), Hispanic (n=124) and white (n=941) patients; with an SLE duration of 1 to <3 years (n=196) and ≥5 years (n=879). Among patients with 1 to <3 years disease duration, the EULAR/ACR criteria had better sensitivity than the ACR criteria (97% vs 81%). The EULAR/ACR criteria performed well in men (sensitivity 93%, specificity 96%) and women (sensitivity 97%, specificity 94%). Among women, the EULAR/ACR criteria had better sensitivity than the ACR criteria (97% vs 83%) and better specificity than the SLICC criteria (94% vs 82%). Among white patients, the EULAR/ACR criteria had better sensitivity than the ACR criteria (95% vs 83%) and better specificity than the SLICC criteria (94% vs 83%). The EULAR/ACR criteria performed well among black patients (sensitivity of 98%, specificity 100%), and had better sensitivity than the ACR criteria among Hispanic patients (100% vs 86%) and Asian patients (97% vs 77%).

Conclusions: The EULAR/ACR 2019 criteria perform well among patients with early disease, men, women, white, black, Hispanic and Asian patients. These criteria have superior sensitivity than the ACR criteria and/or superior specificity than the SLICC criteria across many subgroups.
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http://dx.doi.org/10.1136/annrheumdis-2020-217162DOI Listing
October 2020

Cancer risk in a large inception SLE cohort: Effects of demographics, smoking, and medications.

Arthritis Care Res (Hoboken) 2020 Aug 19. Epub 2020 Aug 19.

Department of Rheumatology, Center for Rheumatology Research Fossvogur, Landspitali University Hospital, Reykjavik, Iceland.

Objective: To assess cancer risk factors in incident SLE.

Methods: Clinical variables and cancer outcomes were assessed annually among incident SLE patients. Multivariate hazard regression models (over-all risk, and most common cancers) included demographics and time-dependent medications (corticosteroids, antimalarial drugs, immunosuppressants), smoking, and adjusted mean SLE Disease Activity Index-2K.

Results: Among 1668 patients (average 9 years follow-up), 65 cancers occurred: 15 breast, 10 non-melanoma skin, seven lung, six hematological, six prostate, five melanoma, three cervical, three renal, two each gastric, head and neck, and thyroid, and one each rectal, sarcoma, thymoma, and uterine cancers. Half of cancers (including all lung cancers) occurred in past/current smokers, versus one-third of patients without cancer. Multivariate analyses indicated over-all cancer risk was related primarily to male sex and older age at SLE diagnosis. In addition, smoking was associated with lung cancer. For breast cancer risk, age was positively and anti-malarial drugs were negatively associated. Anti-malarial drugs and higher disease activity were also negatively associated with non-melanoma skin cancer (NMSC) risk, whereas age and cyclophosphamide were positively associated. Disease activity was associated positively with hematologic and negatively with NMSC risk.

Conclusions: Smoking is a key modifiable risk factor, especially for lung cancer, in SLE. Immunosuppressive medications were not clearly associated with higher risk except for cyclophosphamide and NMSC. Antimalarials were negatively associated with breast cancer and NMSC risk. SLE activity was associated positively with hematologic cancer and negatively with NMSC. Since the absolute number of cancers was small, additional follow-up will help consolidate these findings.
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http://dx.doi.org/10.1002/acr.24425DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7892637PMC
August 2020

Seventy years after Hench's Nobel prize: revisiting the use of glucocorticoids in systemic lupus erythematosus.

Lupus 2020 Sep 15;29(10):1155-1167. Epub 2020 Jun 15.

Department of Women and Children's Health, St Thomas Hospital, London.

In 1950, Hench, Kendall and Reichstein were awarded with the Nobel Prize in Physiology and Medicine for the isolation and first therapeutic use of glucocorticoids. Since then, they have become one of the main agents in the treatment of systemic lupus erythematosus (SLE). The use of high-dose oral glucocorticoids (usually 1 mg/kg/day of prednisone equivalent) have become the rule for treating moderate to severe lupus activity. In addition, tapering schemes have not been well defined, all this leading to prolonged exposures to potentially damaging amounts of glucocorticoids. Several studies have shown that glucocorticoids are a major cause of toxicity in SLE in a dose-dependent manner, with prolonged doses greater than 7.5 mg/day being associated with damage accrual. Thus, there is an urgent need for different therapeutic schedules that can achieve a rapid and durable control of lupus activity while reducing the many unwanted effects of glucocorticoids. Recent data show that pulses of methyl-prednisolone are an effective first-line therapy to treat lupus flares (not only severe ones) without major short or long-term toxicity and allowing a reduction in oral prednisone doses. Universal use of hydroxychloroquine - always recommended, infrequently accomplished - and early therapy with immunosuppressive drugs also help control SLE and reduce prednisone load. Results from observational studies confirm the more rapid achievement of remission and the reduction of long-term damage using these combination schedules with reduced prednisone doses. Seventy years after their first therapeutic use, we are learning to use glucocorticoids in a more efficient and safe manner.
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http://dx.doi.org/10.1177/0961203320930099DOI Listing
September 2020

Accrual of Atherosclerotic Vascular Events in a Multicenter Inception Systemic Lupus Erythematosus Cohort.

Arthritis Rheumatol 2020 10 25;72(10):1734-1740. Epub 2020 Aug 25.

Hairmyres Hospital, East Kilbride, Scotland, UK.

Objective: In previous studies, atherosclerotic vascular events (AVEs) were shown to occur in ~10% of patients with systemic lupus erythematosus (SLE). We undertook this study to investigate the annual occurrence and potential risk factors for AVEs in a multinational, multiethnic inception cohort of patients with SLE.

Methods: A large 33-center cohort of SLE patients was followed up yearly between 1999 and 2017. AVEs were attributed to atherosclerosis based on SLE being inactive at the time of the AVE as well as typical atherosclerotic changes observed on imaging or pathology reports and/or evidence of atherosclerosis elsewhere. Analyses included descriptive statistics, rate of AVEs per 1,000 patient-years, and univariable and multivariable relative risk regression models.

Results: Of the 1,848 patients enrolled in the cohort, 1,710 had ≥1 follow-up visit after enrollment, for a total of 13,666 patient-years. Of these 1,710 patients, 3.6% had ≥1 AVEs attributed to atherosclerosis, for an event rate of 4.6 per 1,000 patient-years. In multivariable analyses, lower AVE rates were associated with antimalarial treatment (hazard ratio [HR] 0.54 [95% confidence interval (95% CI) 0.32-0.91]), while higher AVE rates were associated with any prior vascular event (HR 4.00 [95% CI 1.55-10.30]) and a body mass index of >40 kg/m (HR 2.74 [95% CI 1.04-7.18]). A prior AVE increased the risk of subsequent AVEs (HR 5.42 [95% CI 3.17-9.27], P < 0.001).

Conclusion: The prevalence of AVEs and the rate of AVE accrual demonstrated in the present study is much lower than that seen in previously published data. This may be related to better control of both the disease activity and classic risk factors.
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http://dx.doi.org/10.1002/art.41392DOI Listing
October 2020

Ankle-brachial index and arterial vascular events in systemic lupus erythematosus patients: a 5-year prospective cohort.

Clin Exp Rheumatol 2020 Sep-Oct;38(5):978-984. Epub 2020 Feb 4.

Department of Internal Medicine, Hospital Universitario Cruces, University of The Basque Country, Bizkaia, Spain.

Objectives: To determine the potential predictive value in patients with systemic lupus erythematous of the ankle-brachial index (ABI) for the occurrence of arterial vascular events.

Methods: 216 lupus patients from a prospective clinical cohort were evaluated using the ABI at the start of the study and then followed up for 5 years. Abnormal ABI was defined as an index ≤0.9 or >1.4. Several potential vascular risk factors were also evaluated. Arterial vascular events (AVE): coronary events, cerebrovascular events, peripheral arterial disease and death related to vascular disease. Survival analysis was performed using a competitive risk regression approach, considering non-vascular death as a competitive event.

Results: 18 arterial events and 14 deaths were identified. In the competitive risk regression analysis, independent predictors of higher risk were identified: family history of early AVE [subdistribution hazard ratio (SHR) 5.44, 95% confidence interval (CI) 1.69-17.50, p=0.004)], cumulative prednisone (grams) (SHR 1.01, 95% CI 1.01-1.03, p=0.007) and a personal history of arterial thrombosis (SHR 5.44, 95% CI 1.45-14.59, p=0.004). Female gender was a protective factor (SHR 0.22, 95% CI 0.07-0.77, p=0.017). A statistical trend was detected with abnormal ABI (SHR 2.65, 95% CI 0.86-8.14, p=0.089).

Conclusions: Male gender, exposure to high cumulative doses of prednisone, family history of early arterial vascular disease and occurrence of previous arterial thrombosis are independent risk predictors of arterial vascular events in patients with systemic lupus erythematosus. Abnormal ABI may be related to high risk for arterial vascular events.
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October 2020

Comparative study between two European inception cohorts of patients with early systemic lupus erythematosus.

Clin Exp Rheumatol 2020 Sep-Oct;38(5):925-932. Epub 2020 Jan 20.

Rheumatology Unit, Azienda Ospedaliera San Camillo-Forlanini, Roma, Italy.

Objectives: To compare the main characteristics of two inception cohorts (Italian [ITC] and Spanish [SPC]) cohorts of patients with systemic lupus erythematosus (SLE) at the time of diagnosis and at one year of follow-up.

Methods: Demographic, clinical and immunological characteristics, and treatments at SLE diagnosis and at 12 months of follow-up of ITC and SPC were compared.

Results: One hundred and sixty-four patients in the ITC and 231 patients in the SPC were compared. the patients from ITC were younger at SLE diagnosis (41.1±15.0 years vs. 46.4±15.6 years; p<0.001) and had a higher prevalence of arthritis (62.8% vs. 45.5%; p=0.001), serositis (25.6% vs. 16.0%; p=0.026), neurological involvement (7.9% vs. 1.7%; p=0.006), and immunological abnormalities (anti-dsDNA, anti-Sm, antiphospholipid antibodies) (93.9% vs. 77.8%; p<0.001). Conversely, photosensitivity (29.5% in ITC vs. 45.9% in SPC; p=0.001) and oral ulcers (12.4% vs. 30.3%; p<0.001) were more frequent at onset of SLE in the Spanish patients. At the first 12 months of follow-up, these differences were maintained. At SLE onset, more Italian patients received glucocorticoids (85.4% vs. 50.2%; p<0.001) and immunosuppressive agents. At 12 months of follow-up, more Spanish patients were treated with antimalarials (75.6% in ITC vs. 90.0% in SPC; p<0.001). Conversely, the use of glucocorticoids was lower in SPC (89.0% in ITC vs. 57.1% in SPC; p<0.001).

Conclusions: These cohorts presented different profiles in terms of pattern of organ/system involvement and disease treatment, possibly as a consequence of patient selection or different disease management approaches between Italy and Spain.
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October 2020

Neuropsychiatric events in systemic lupus erythematosus: a longitudinal analysis of outcomes in an international inception cohort using a multistate model approach.

Ann Rheum Dis 2020 03 8;79(3):356-362. Epub 2020 Jan 8.

Copenhagen Lupus and Vasculitis Clinic, 4242, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.

Objectives: Using a reversible multistate model, we prospectively examined neuropsychiatric (NP) events for attribution, outcome and association with health-related quality of life (HRQoL), in an international, inception cohort of systemic lupus erythematosus (SLE) patients.

Methods: Annual assessments for 19 NP events attributed to SLE and non-SLE causes, physician determination of outcome and patient HRQoL (short-form (SF)-36 scores) were measured. Time-to-event analysis and multistate modelling examined the onset, recurrence and transition between NP states.

Results: NP events occurred in 955/1827 (52.3%) patients and 592/1910 (31.0%) unique events were attributed to SLE. In the first 2 years of follow-up the relative risk (95% CI) for SLE NP events was 6.16 (4.96, 7.66) and non-SLE events was 4.66 (4.01, 5.43) compared with thereafter. Patients without SLE NP events at initial assessment had a 74% probability of being event free at 10 years. For non-SLE NP events the estimate was 48%. The majority of NP events resolved over 10 years but mortality was higher in patients with NP events attributed to SLE (16%) versus patients with no NPSLE events (6%) while the rate was comparable in patients with non-SLE NP events (7%) compared with patients with no non-SLE events (6%). Patients with NP events had lower SF-36 summary scores compared with those without NP events and resolved NP states (p<0.001).

Conclusions: NP events occur most frequently around the diagnosis of SLE. Although the majority of events resolve they are associated with reduced HRQoL and excess mortality. Multistate modelling is well suited for the assessment of NP events in SLE.
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http://dx.doi.org/10.1136/annrheumdis-2019-216150DOI Listing
March 2020

Pregnancy control in Patients with Systemic Lupus Erythematosus/Antiphospholipid Syndrome. Part 3: Childbirth. Puerperium. Breastfeeding Contraception. Newborn.

Reumatol Clin (Engl Ed) 2021 Apr 10;17(4):183-186. Epub 2019 Dec 10.

Servicio de Obstetricia y Ginecología, Hospital Universitario La Paz, Madrid, España.

Objective: In order to agree on the fundamental aspects related to the management of pregnancy in patients with systemic lupus erythematosus and antiphospholipid syndrome, the Spanish Societies of Gynaecology and Obstetrics, Internal Medicine and Rheumatology have set up a working group for the preparation of three consensus documents.

Methods: Each of the Scientific Societies involved proposed five representatives based on their experience in the field of pregnancy control in patients with autoimmune diseases. The recommendations were developed following the Delphi methodology.

Results: This third document contains the recommendations regarding the management of delivery, puerperium and lactation, including medication use during these periods and the care of the newborn. In addition, a section on contraception is included.

Conclusions: These multidisciplinary recommendations are considered decision-making tools for clinicians involved in the care of patients with systemic lupus erythematosus/antiphospholipid syndrome during pregnancy.
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http://dx.doi.org/10.1016/j.reuma.2019.09.004DOI Listing
April 2021

Pregnancy Control in Patients with Systemic Lupus Erythematosus and Antiphospholipid Syndrome. Part 1: Infertility, Ovarian Preservation and Preconception Assessment. Consensus Document of the Spanish Society of Gynaecology and Obstetrics (SEGO), the Spanish Society of Internal Medicine (SEMI) and the Spanish Society of Rheumatology (SER).

Reumatol Clin (Engl Ed) 2021 Feb 26;17(2):61-66. Epub 2019 Nov 26.

Servicio de Reumatología, Hospital Universitario Marqués de Valdecilla, IDIVAL, Facultad de Medicina, Universidad de Cantabria, Santander, España. Electronic address:

Objective: Pregnancy and puerperium are considered a risk situation in women with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). Therefore, specialized assessment is essential both preconception and during pregnancy and the puerperium. Likewise, it is very important that different specialists in autoimmune diseases and high-risk pregnancies participate in the follow-up of these patients in a coordinated manner. The Spanish Society of Gynaecology and Obstetrics, the Spanish Society of Internal Medicine, and the Spanish Society of Rheumatology have set up a working group for the preparation of three consensus documents.

Methods: The stages of the work were: distribution of work in three groups corresponding to the three periods related to pregnancy (preconception, during pregnancy and childbirth and puerperium), identification of key areas, exhaustive review of the literature and formulation of recommendations.

Results: This first document includes the 48 recommendations that address aspects related to infertility, the need for and treatments for gonadal preservation and preconception assessment.

Conclusions: These multidisciplinary recommendations are considered decision-making tools for clinicians involved in the care of patients with SLE/APS during pregnancy.
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http://dx.doi.org/10.1016/j.reuma.2019.09.002DOI Listing
February 2021

Pregnancy control in patients with systemic lupus erythematosus/antiphospholipid syndrome. Part 2: Pregnancy follow-up.

Reumatol Clin (Engl Ed) 2021 Mar 25;17(3):125-131. Epub 2019 Nov 25.

Unidad de Enfermedades Autoinmunes, Servicio de Medicina Interna, BioCruces Bizkaia Health Research Institute, Hospital Universitario Cruces, UPV/EHU, Barakaldo, Bizkaia, España. Electronic address:

Objective: In order to agree on the fundamental aspects related to the management of pregnancy in patients with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS), the Spanish Societies of Gynaecology and Obstetrics, Internal Medicine and Rheumatology set up a working group for the preparation of three consensus documents.

Methods: Each of the Scientific Societies involved proposed five representatives based on their experience in the field of pregnancy control in patients with autoimmune diseases. The recommendations were developed following the Delphi methodology.

Results: This second document contains the recommendations regarding the management of pregnancy in women with SLE and APS, including complications such as lupus activity, congenital heart block, thrombotic and obstetric manifestations of APS and placental vascular disease.

Conclusions: These multidisciplinary recommendations are considered decision-making tools for clinicians involved in the care of patients with SLE/APS during pregnancy.
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http://dx.doi.org/10.1016/j.reuma.2019.09.003DOI Listing
March 2021

Prediction of Damage Accrual in Systemic Lupus Erythematosus Using the Systemic Lupus International Collaborating Clinics Frailty Index.

Arthritis Rheumatol 2020 04 12;72(4):658-666. Epub 2020 Feb 12.

Emory University School of Medicine, Atlanta, Georgia.

Objective: The Systemic Lupus International Collaborating Clinics (SLICC) frailty index (FI) has been shown to predict mortality, but its association with other important outcomes is unknown. We examined the association of baseline SLICC FI values with damage accrual in the SLICC inception cohort.

Methods: The baseline visit was defined as the first visit at which both organ damage (SLICC/American College of Rheumatology Damage Index [SDI]) and health-related quality of life (Short Form 36) were assessed. Baseline SLICC FI scores were calculated. Damage accrual was measured by the increase in SDI between the baseline assessment and the last study visit. Multivariable negative binomial regression was used to estimate the association between baseline SLICC FI values and the rate of increase in the SDI during follow-up, adjusting for relevant demographic and clinical characteristics.

Results: The 1,549 systemic lupus erythematosus (SLE) patients eligible for this analysis were mostly female (88.7%) with a mean ± SD age of 35.7 ± 13.3 years and a median disease duration of 1.2 years (interquartile range 0.9-1.5 years) at baseline. The mean ± SD baseline SLICC FI was 0.17 ± 0.08. Over a mean ± SD follow-up of 7.2 ± 3.7 years, 653 patients (42.2%) had an increase in SDI. Higher baseline SLICC FI values (per 0.05 increase) were associated with higher rates of increase in the SDI during follow-up (incidence rate ratio [IRR] 1.19 [95% confidence interval 1.13-1.25]), after adjusting for age, sex, ethnicity/region, education, baseline SLE Disease Activity Index 2000, baseline SDI, and baseline use of glucocorticoids, antimalarials, and immunosuppressive agents.

Conclusion: Our findings indicate that the SLICC FI predicts damage accrual in incident SLE, which further supports the SLICC FI as a valid health measure in SLE.
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http://dx.doi.org/10.1002/art.41144DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7113092PMC
April 2020

Soluble urokinase plasminogen activator receptor (suPAR) levels predict damage accrual in patients with recent-onset systemic lupus erythematosus.

J Autoimmun 2020 01 17;106:102340. Epub 2019 Oct 17.

Department of Clinical Sciences Lund, Section of Rheumatology, Lund University, Lund, Sweden.

Objective: The soluble urokinase plasminogen activator receptor (suPAR) has potential as a prognosis and severity biomarker in several inflammatory and infectious diseases. In a previous cross-sectional study, suPAR levels were shown to reflect damage accrual in cases of systemic lupus erythematosus (SLE). Herein, we evaluated suPAR as a predictor of future organ damage in recent-onset SLE.

Methods: Included were 344 patients from the Systemic Lupus International Collaborating Clinics (SLICC) Inception Cohort who met the 1997 American College of Rheumatology classification criteria with 5-years of follow-up data available. Baseline sera from patients and age- and sex-matched controls were assayed for suPAR. Organ damage was assessed annually using the SLICC/ACR damage index (SDI).

Results: The levels of suPAR were higher in patients who accrued damage, particularly those with SDI≥2 at 5 years (N = 32, 46.8% increase, p = 0.004), as compared to patients without damage. Logistic regression analysis revealed a significant impact of suPAR on SDI outcome (SDI≥2; OR = 1.14; 95% CI 1.03-1.26), also after adjustment for confounding factors. In an optimized logistic regression to predict damage, suPAR persisted as a predictor, together with baseline disease activity (SLEDAI-2K), age, and non-Caucasian ethnicity (model AUC = 0.77). Dissecting SDI into organ systems revealed higher suPAR levels in patients who developed musculoskeletal damage (SDI≥1; p = 0.007).

Conclusion: Prognostic biomarkers identify patients who are at risk of acquiring early damage and therefore need careful observation and targeted treatment strategies. Overall, suPAR constitutes an interesting biomarker for patient stratification and for identifying SLE patients who are at risk of acquiring organ damage during the first 5 years of disease.
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http://dx.doi.org/10.1016/j.jaut.2019.102340DOI Listing
January 2020

Economic Evaluation of Damage Accrual in an International Systemic Lupus Erythematosus Inception Cohort Using a Multistate Model Approach.

Arthritis Care Res (Hoboken) 2020 12;72(12):1800-1808

Hairmyres Hospital, East Kilbride, Scotland, UK.

Objective: There is a paucity of data regarding health care costs associated with damage accrual in systemic lupus erythematosus. The present study was undertaken to describe costs associated with damage states across the disease course using multistate modeling.

Methods: Patients from 33 centers in 11 countries were enrolled in the Systemic Lupus International Collaborating Clinics (SLICC) inception cohort within 15 months of diagnosis. Annual data on demographics, disease activity, damage (SLICC/American College of Rheumatology Damage Index [SDI]), hospitalizations, medications, dialysis, and selected procedures were collected. Ten-year cumulative costs (Canadian dollars) were estimated by multiplying annual costs associated with each SDI state by the expected state duration using a multistate model.

Results: A total of 1,687 patients participated; 88.7% were female, 49.0% were white, mean ± SD age at diagnosis was 34.6 ± 13.3 years, and mean time to follow-up was 8.9 years (range 0.6-18.5 years). Mean annual costs were higher for those with higher SDI scores as follows: $22,006 (Canadian) (95% confidence interval [95% CI] $16,662, $27,350) for SDI scores ≥5 versus $1,833 (95% CI $1,134, $2,532) for SDI scores of 0. Similarly, 10-year cumulative costs were higher for those with higher SDI scores at the beginning of the 10-year interval as follows: $189,073 (Canadian) (95% CI $142,318, $235,827) for SDI scores ≥5 versus $21,713 (95% CI $13,639, $29,788) for SDI scores of 0.

Conclusion: Patients with the highest SDI scores incur 10-year cumulative costs that are ~9-fold higher than those with the lowest SDI scores. By estimating the damage trajectory and incorporating annual costs, data on damage can be used to estimate future costs, which is critical knowledge for evaluating the cost-effectiveness of novel therapies.
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http://dx.doi.org/10.1002/acr.24092DOI Listing
December 2020

Peripheral Nervous System Disease in Systemic Lupus Erythematosus: Results From an International Inception Cohort Study.

Arthritis Rheumatol 2020 01 28;72(1):67-77. Epub 2019 Nov 28.

Amsterdam University Medical Centers, Amsterdam, The Netherlands.

Objective: To determine the frequency, clinical characteristics, associations, and outcomes of different types of peripheral nervous system (PNS) disease in a multiethnic/multiracial, prospective inception cohort of systemic lupus erythematosus (SLE) patients.

Methods: Patients were evaluated annually for 19 neuropsychiatric (NP) events including 7 types of PNS disease. SLE disease activity, organ damage, autoantibodies, and patient and physician assessment of outcome were measured. Time to event and linear regressions were used as appropriate.

Results: Of 1,827 SLE patients, 88.8% were female, and 48.8% were white. The mean ± SD age was 35.1 ± 13.3 years, disease duration at enrollment was 5.6 ± 4.2 months, and follow-up was 7.6 ± 4.6 years. There were 161 PNS events in 139 (7.6%) of 1,827 patients. The predominant events were peripheral neuropathy (66 of 161 [41.0%]), mononeuropathy (44 of 161 [27.3%]), and cranial neuropathy (39 of 161 [24.2%]), and the majority were attributed to SLE. Multivariate Cox regressions suggested longer time to resolution in patients with a history of neuropathy, older age at SLE diagnosis, higher SLE Disease Activity Index 2000 scores, and for peripheral neuropathy versus other neuropathies. Neuropathy was associated with significantly lower Short Form 36 (SF-36) physical and mental component summary scores versus no NP events. According to physician assessment, the majority of neuropathies resolved or improved over time, which was associated with improvements in SF-36 summary scores for peripheral neuropathy and mononeuropathy.

Conclusion: PNS disease is an important component of total NPSLE and has a significant negative impact on health-related quality of life. The outcome is favorable for most patients, but our findings indicate that several factors are associated with longer time to resolution.
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http://dx.doi.org/10.1002/art.41070DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6935421PMC
January 2020

2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus.

Arthritis Rheumatol 2019 09 6;71(9):1400-1412. Epub 2019 Aug 6.

Azienda Ospedaliero Universitaria Pisana, University of Pisa, Pisa, Italy.

Objective: To develop new classification criteria for systemic lupus erythematosus (SLE) jointly supported by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR).

Methods: This international initiative had four phases. 1) Evaluation of antinuclear antibody (ANA) as an entry criterion through systematic review and meta-regression of the literature and criteria generation through an international Delphi exercise, an early patient cohort, and a patient survey. 2) Criteria reduction by Delphi and nominal group technique exercises. 3) Criteria definition and weighting based on criterion performance and on results of a multi-criteria decision analysis. 4) Refinement of weights and threshold scores in a new derivation cohort of 1,001 subjects and validation compared with previous criteria in a new validation cohort of 1,270 subjects.

Results: The 2019 EULAR/ACR classification criteria for SLE include positive ANA at least once as obligatory entry criterion; followed by additive weighted criteria grouped in 7 clinical (constitutional, hematologic, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and 3 immunologic (antiphospholipid antibodies, complement proteins, SLE-specific antibodies) domains, and weighted from 2 to 10. Patients accumulating ≥10 points are classified. In the validation cohort, the new criteria had a sensitivity of 96.1% and specificity of 93.4%, compared with 82.8% sensitivity and 93.4% specificity of the ACR 1997 and 96.7% sensitivity and 83.7% specificity of the Systemic Lupus International Collaborating Clinics 2012 criteria.

Conclusion: These new classification criteria were developed using rigorous methodology with multidisciplinary and international input, and have excellent sensitivity and specificity. Use of ANA entry criterion, hierarchically clustered, and weighted criteria reflects current thinking about SLE and provides an improved foundation for SLE research.
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http://dx.doi.org/10.1002/art.40930DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6827566PMC
September 2019
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