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      Long-term outcomes in systemic lupus erythematosus: trends over time and major contributors

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          Abstract

          SLE is a chronic autoimmune rheumatic disorder of high heterogeneity in clinical presentation, treatment response and prognosis. Long-term outcomes in SLE have been dramatically improved over the past decades, however, increased morbidity and mortality, especially among young individuals, still exists. Unmet needs include residual disease activity and frequent flares, glucocorticoid treatment dependency and toxicity, comorbidity burden, reduced health-related quality of life, health disparities and damage. The main determinants of long-term outcomes in SLE are age, sex, race/ethnicity, genetic profile, environmental factors including smoking, disease activity, major organ involvement such as lupus nephritis and CNS involvement, comorbidities including cardiovascular disease and serious infections, coexistence with APS, treatment adherence, socio-economic factors and access to care. In this review we discuss trends in long-term outcomes in SLE over the years and major contributors such as genetic, disease-related, treatment, comorbidity, socio-economic and other factors.

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          2019 update of the EULAR recommendations for the management of systemic lupus erythematosus

          Antonis Fanouriakis, Myrto Kostopoulou, Alessia Alunno … (2019)
          Our objective was to update the EULAR recommendations for the management of systemic lupus erythematosus (SLE), based on emerging new evidence. We performed a systematic literature review (01/2007–12/2017), followed by modified Delphi method, to form questions, elicit expert opinions and reach consensus. Treatment in SLE aims at remission or low disease activity and prevention of flares. Hydroxychloroquine is recommended in all patients with lupus, at a dose not exceeding 5 mg/kg real body weight. During chronic maintenance treatment, glucocorticoids (GC) should be minimised to less than 7.5 mg/day (prednisone equivalent) and, when possible, withdrawn. Appropriate initiation of immunomodulatory agents (methotrexate, azathioprine, mycophenolate) can expedite the tapering/discontinuation of GC. In persistently active or flaring extrarenal disease, add-on belimumab should be considered; rituximab (RTX) may be considered in organ-threatening, refractory disease. Updated specific recommendations are also provided for cutaneous, neuropsychiatric, haematological and renal disease. Patients with SLE should be assessed for their antiphospholipid antibody status, infectious and cardiovascular diseases risk profile and preventative strategies be tailored accordingly. The updated recommendations provide physicians and patients with updated consensus guidance on the management of SLE, combining evidence-base and expert-opinion.
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            EULAR recommendations for the management of antiphospholipid syndrome in adults

            Maria G Tektonidou, Laura Andreoli, Marteen Limper … (2019)
            The objective was to develop evidence-based recommendations for the management of antiphospholipid syndrome (APS) in adults. Based on evidence from a systematic literature review and expert opinion, overarching principles and recommendations were formulated and voted. High-risk antiphospholipid antibody (aPL) profile is associated with greater risk for thrombotic and obstetric APS. Risk modification includes screening for and management of cardiovascular and venous thrombosis risk factors, patient education about treatment adherence, and lifestyle counselling. Low-dose aspirin (LDA) is recommended for asymptomatic aPL carriers, patients with systemic lupus erythematosus without prior thrombotic or obstetric APS, and non-pregnant women with a history of obstetric APS only, all with high-risk aPL profiles. Patients with APS and first unprovoked venous thrombosis should receive long-term treatment with vitamin K antagonists (VKA) with a target international normalised ratio (INR) of 2–3. In patients with APS with first arterial thrombosis, treatment with VKA with INR 2–3 or INR 3–4 is recommended, considering the individual’s bleeding/thrombosis risk. Rivaroxaban should not be used in patients with APS with triple aPL positivity. For patients with recurrent arterial or venous thrombosis despite adequate treatment, addition of LDA, increase of INR target to 3–4 or switch to low molecular weight heparin may be considered. In women with prior obstetric APS, combination treatment with LDA and prophylactic dosage heparin during pregnancy is recommended. In patients with recurrent pregnancy complications, increase of heparin to therapeutic dose, addition of hydroxychloroquine or addition of low-dose prednisolone in the first trimester may be considered. These recommendations aim to guide treatment in adults with APS. High-quality evidence is limited, indicating a need for more research.
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              2019 update of EULAR recommendations for vaccination in adult patients with autoimmune inflammatory rheumatic diseases

              Victoria Furer, Christien Rondaan, Marloes Heijstek … (2019)
              To update the European League Against Rheumatism (EULAR) recommendations for vaccination in adult patients with autoimmune inflammatory rheumatic diseases (AIIRD) published in 2011. Four systematic literature reviews were performed regarding the incidence/prevalence of vaccine-preventable infections among patients with AIIRD; efficacy, immunogenicity and safety of vaccines; effect of anti-rheumatic drugs on the response to vaccines; effect of vaccination of household of AIIRDs patients. Subsequently, recommendations were formulated based on the evidence and expert opinion. The updated recommendations comprise six overarching principles and nine recommendations. The former address the need for an annual vaccination status assessment, shared decision-making and timing of vaccination, favouring vaccination during quiescent disease, preferably prior to the initiation of immunosuppression. Non-live vaccines can be safely provided to AIIRD patients regardless of underlying therapy, whereas live-attenuated vaccines may be considered with caution. Influenza and pneumococcal vaccination should be strongly considered for the majority of patients with AIIRD. Tetanus toxoid and human papilloma virus vaccination should be provided to AIIRD patients as recommended for the general population. Hepatitis A, hepatitis B and herpes zoster vaccination should be administered to AIIRD patients at risk. Immunocompetent household members of patients with AIIRD should receive vaccines according to national guidelines, except for the oral poliomyelitis vaccine. Live-attenuated vaccines should be avoided during the first 6 months of life in newborns of mothers treated with biologics during the second half of pregnancy. These 2019 EULAR recommendations provide an up-to-date guidance on the management of vaccinations in patients with AIIRD.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Rheumatology (Oxford)
                Journal ID (iso-abbrev): Rheumatology (Oxford)
                Journal ID (publisher-id): brheum
                Title: Rheumatology (Oxford, England)
                Publisher: Oxford University Press
                ISSN (Print): 1462-0324
                ISSN (Electronic): 1462-0332
                Publication date Collection: December 2020
                Publication date (Electronic): 05 December 2020
                Publication date PMC-release: 05 December 2020
                Volume: 59
                Issue: Suppl 5 , Lupus in the 21st Century
                Pages: v29-v38
                Affiliations
                [k1 ] Department of Rheumatology, Hôpitaux Universitaires de Strasbourg, INSERM UMR-S 1109, Centre National de Référence des Maladies Auto-immunes Systémiques Rares (RESO) , Strasbourg, France
                [k2 ] Rheumatology Unit, First Department of Propaedeutic Internal Medicine, Medical School, National and Kapodistrian University of Athens, Joint Rheumatology Program, Laiko Hospital , Athens, Greece
                Author notes
                Correspondence to: Maria G. Tektonidou, Head of Rheumatology Unit, First Department of Propaedeutic Internal Medicine, Medical School, National and Kapodistrian University of Athens, Laiko Hospital, 17 Agiou Thoma Str., 11527 Athens, Greece. E-mail: mtektonidou@ 123456gmail.com
                Author information
                http://orcid.org/0000-0003-2238-0975
                Article
                Publisher ID: keaa382
                DOI: 10.1093/rheumatology/keaa382
                PMC ID: 7719040
                PubMed ID: 33280012
                SO-VID: 39a79af9-e1fb-41bb-bcc0-fac33f2ada8f
                Copyright © © The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology.
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com

                History
                Date received : 25 March 2020
                Date revision received : 03 June 2020
                Page count
                Pages: 10
                Funding
                Funded by: GSK, DOI 10.13039/100004330;
                Funded by: AstraZeneca, DOI 10.13039/100004325;
                Categories
                Subject: Supplement Papers
                Subject: AcademicSubjects/MED00360

                ScienceOpen disciplines: Rheumatology
                Keywords: sle,long-term outcomes,lupus nephritis,damage,mortality,comorbidities,glucocorticoids,treatment adherence,socio-economic factors,smoking
                Data availability:
                ScienceOpen disciplines: Rheumatology
                Keywords: sle, long-term outcomes, lupus nephritis, damage, mortality, comorbidities, glucocorticoids, treatment adherence, socio-economic factors, smoking

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