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  • University of Birmingham

    2019 update of the EULAR recommendations for the management of systemic lupus erythematosus Fanouriakis, Antonis; Gordon, Caroline

    DOI: 10.1136/annrheumdis-2019-215089

    License: None: All rights reserved

    Document Version Peer reviewed version

    Citation for published version (Harvard): Fanouriakis, A & Gordon, C 2019, '2019 update of the EULAR recommendations for the management of systemic lupus erythematosus', Annals of the Rheumatic Diseases, vol. 78, no. 6, pp. 736-745. https://doi.org/10.1136/annrheumdis-2019-215089

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  •   1

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

    Antonis Fanouriakis1*, Myrto Kostopoulou2, Alessia Alunno3, Martin Aringer4, Ingeborg Bajema5, John N.

    Boletis6, Ricard Cervera7, Andrea Doria8, Caroline Gordon9, Marcello Govoni10, Frederic Houssiau11, David

    Jayne12, Marios Kouloumas13, Annegret Kuhn14, Janni L. Larsen15, Kirsten Lerstrom16, Gabriela Moroni17,

    Marta Mosca18, Matthias Schneider19, Josef S. Smolen20, Elisabet Svenungsson21, Vladimir Tesar22, Angela

    Tincani23, Anne Troldborg24, Ronald van Vollenhoven25, Jörg Wenzel26, George Bertsias27**, Dimitrios T.

    Boumpas1,28,29**

    1 Rheumatology and Clinical Immunology Unit, "Attikon" University Hospital, Athens, Greece

    2 Department of Nephrology, "G. Gennimatas" General Hospital, Athens, Greece

    3 Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy

    4 Division of Rheumatology, Department of Medicine III, University Medical Center & Faculty of Medicine

    Carl Gustav Carus at the TU Dresden, Dresden, Germany

    5 Department of Pathology, Leiden University Medical Center, Leiden, Netherlands

    6 Nephrology Department and Renal Transplantation Unit, "Laikon" Hospital, National and Kapodistrian

    University of Athens, Medical School, Athens, Greece

    7 Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain

    8 Rheumatology Unit, Department of Medicine, University of Padova, Padova, Italy

    9 Rheumatology Research Group, Institute of Inflammation and Ageing, University of Birmingham,

    Birmingham, United Kingdom

    10 Department of Medical Sciences, Section of Rheumatology, University of Ferrara, Azienda Ospedaliero-

    Universitaria Sant'Anna Ferrara, Italy

    11 Cliniques Universitaires Saint-Luc, Université catholique de Louvain, Brussels, Belgium

  •   2

    12 Department of Medicine, Cambridge, Cambridge, United Kingdom

    13 Cyprus League Against Rheumatism, Aglantzia, Cyprus

    14 University Hospital Muenster, Muenster, Germany

    15 Copenhagen Lupus and Vasculitis Clinic, Rheumatology and Spine Diseases Centre, Rigshospitalet,

    Copenhagen, Denmark

    16 Lupus Europe, Farum, Denmark

    17 Nephrology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan

    18 Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy

    19 Department of Rheumatology & Hiller Research Unit Rheumatology, UKD, Heinrich-Heine University,

    Düsseldorf, Germany

    20 Division of Rheumatology, Department of Medicine 3, Medical University of Vienna, Vienna, Austria

    21 Department of Medicine, Rheumatology Unit, Karolinska Institutet and Karolinska University Hospital,

    Stockholm, Sweden

    22 Department of Nephrology, 1st Faculty of Medicine and General University Hospital, Charles University,

    Prague, Czech Republic

    23 Rheumatology and Clinical Immunology, University of Brescia, Brescia, Italy

    24 Department of Rheumatology, Aarhus University Hospital, Aarhus, Denmark

    25 Department of Rheumatology and Clinical Immunology, Amsterdam University Medical Centers,

    Amsterdam, The Netherlands

    26 Department of Dermatology and Allergy, University Hospital Bonn, Germany

    27 Rheumatology, Clinical Immunology and Allergy, University Hospital of Heraklion, Greece

    28 Laboratory of Autoimmunity and Inflammation, Biomedical Research Foundation of the Academy of Athens,

    Athens, Greece

  •   3

    29 Joint Academic Rheumatology Program, Medical School, National and Kapodestrian University of Athens,

    Athens, Greece and Medical School, University of Cyprus, Nicosia, Cyprus

    * Antonis Fanouriakis was supported by an Articulum Fellowship and a grant from the Hellenic Society of

    Rheumatology during the completion of this work

    ** These authors contributed equally to this work

    Corresponding author: Dimitrios Boumpas, MD, FACP

    Professor of Medicine

    National and Kapodestrian University of Athens

    12462 Athens, Greece

    Tel.: (+30) 210 5831990

    E-mail: boumpasd@uoc.gr

    Word count: 3612

  •   4

    Abstract

    Objectives: To update the EULAR recommendations for the management of systemic lupus erythematosus

    (SLE) based upon emerging new evidence.

    Methods: Systematic literature review (01/2007-12/2017) followed by modified Delphi method to form

    questions, elicit expert opinions and reach consensus.

    Results: Treatment in SLE aims at remission or low disease activity and prevention of flares.

    Hydroxychloroquine is recommended in all lupus patients, at a dose not exceeding 5mg/kg real body weight.

    During chronic maintenance treatment, glucocorticoids should be minimized 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 glucocorticoids. In

    persistently active or flaring extrarenal disease, add-on belimumab should be considered; rituximab may be

    considered in organ-threatening, refractory disease. Updated specific recommendations are also provided for

    cutaneous, neuropsychiatric, haematological and renal disease. SLE patients should be assessed for their

    antiphospholipid antibody status, infectious and cardiovascular diseases risk profile, and preventative

    strategies be tailored accordingly.

    Conclusion: The recommendations provide physicians and patients with updated consensus guidance on the

    management of SLE, combining evidence-base and expert-opinion.

    Keywords: Systemic lupus erythematosus, treatment, lupus nephritis

  •   5

    Introduction

    Systemic lupus erythematosus (SLE) has variable presentation, course and prognosis. The wide acceptance

    and popularity of the first EULAR recommendations for its management, published in 2008,1 promp

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