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2019 update of the EULAR recommendations for the management of systemic lupus erythematosus Fanouriakis, Antonis; Gordon, Caroline
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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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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.
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
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,
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,
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,
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,
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
Word count: 3612
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
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