ERN ReCONNET

Disease Info

Disease: Systemic Lupus Erythematosus

Systemic Lupus Erythematosus (SLE)

SLE is the paradigm of systemic autoimmune diseases characterized by a wide spectrum of clinical manifestations with an unpredictable relapsing-remitting course. SLE typically affects young women between 16 and 55 years and may involve almost all organs and tissues.

Some of the most common clinical features are: mucocutaneous lesions, articular manifestations, renal involvement, haematological disorders, serositis and fever. 40-70% of SLE patients present lupus nephritis (LN) whose dominant feature is proteinuria usually associated with urinary sediment abnormalities. In most severe cases LN may result in chronic renal failure.

Neuropsychiatric manifestations can also be present and they include several symptoms such as headache, seizure disorder, psychosis, acute confusional state and cognitive dysfunction. A higher rate of mortality and morbidity is described among patients with renal and neuropsychiatric involvement.

The serological picture of SLE is characterized by the positivity of many autoantibodies among which the most specific are anti-dsDNA and anti-Sm. The presence of antiphospholipid (aPL) antibodies in all SLE patients is associated with a worse prognosis. During SLE course patients may develop both disease- and treatment-related damage; although the better use of the available therapies has greatly improved SLE patients’ outcome, the disease is still associated with a significant morbidity.

Laurent Arnaud
Disease Coordinator

Jeanette Andersen
ePAG representative

Maarten Limper
Disease Coordinator

Alain Cornet
ePAG representative

Laurent Arnaud, Drug-induced lupus. From historical case reports to big data

Victoria Werth, Treatment of cutaneous manifestations in SLE

Guillermo Ruiz-Irastorza, Glucocorticoids in SLE. Can they be used differently?

Patients’ organisations

LIST OF USEFUL REFERENCES

2021 DORIS definition of remission in SLE: final recommendations from an international task force
van Vollenhoven RF, Bertsias G, Doria A, Isenberg D, Morand E, Petri MA, Pons-Estel BA, Rahman A, Ugarte-Gil MF, Voskuyl A, Arnaud L, Bruce IN, Cervera R, Costedoat-Chalumeau N, Gordon C, Houssiau FA, Mosca M, Schneider M, Ward MM, Alarcon G, Aringer M, Askenase A, Bae SC, Bootsma H, Boumpas DT, Brunner H, Clarke AE, Coney C, Czirják L, Dörner T, Faria R, Fischer R, Fritsch-Stork R, Inanc M, Jacobsen S, Jayne D, Kuhn A, van Leeuw B, Limper M, Mariette X, Navarra S, Nikpour M, Olesinska MH, Pons-Estel G, Romero-Diaz J, Rubio B, Schoenfeld Y, Bonfá E, Smolen J, Teng YKO, Tincani A, Tsang-A-Sjoe M, Vasconcelos C, Voss A, Werth VP, Zakharhova E, Aranow C.
Lupus Sci Med. 2021 Nov;8(1):e000538.

Safety management in treatment with antimalarials in rheumatology. Interdisciplinary recommendations on the basis of a systematic literature review
Fiehn C, Ness T, Weseloh C, Specker C, Hadjiski D, Detert J, Krüger K; DGRh Kommission Pharmakotherapie.
Z Rheumatol. 2021 Feb;80(Suppl 1):1-9.

Neonatal lupus erythematosus – practical guidelines
Derdulska JM, Rudnicka L, Szykut-Badaczewska A, Mehrholz D, Nowicki RJ, Barańska-Rybak W, Wilkowska A.
J Perinat Med. 2021 Jan 18;49(5):529-538.

2019 update of the EULAR recommendations for the management of systemic lupus erythematosus
Fanouriakis A, Kostopoulou M, Alunno A, Aringer M, Bajema I, Boletis JN, Cervera R, Doria A, Gordon C, Govoni M, Houssiau F, Jayne D, Kouloumas M, Kuhn A, Larsen JL, Lerstrøm K, Moroni G, Mosca M, Schneider M, Smolen JS, Svenungsson E, Tesar V, Tincani A, Troldborg A, van Vollenhoven R, Wenzel J, Bertsias G, Boumpas DT.
Ann Rheum Dis. 2019 Jun;78(6):736-745.

The British Society for Rheumatology guideline for the management of systemic lupus erythematosus in adults
Gordon C, Amissah-Arthur MB, Gayed M, Brown S, Bruce IN, D’Cruz D, Empson B, Griffiths B, Jayne D, Khamashta M, Lightstone L, Norton P, Norton Y, Schreiber K, Isenberg D; British Society for Rheumatology Standards, Audit and Guidelines Working Group.
Rheumatology (Oxford). 2018 Jan 1;57(1):e1-e45.

European evidence-based recommendations for the diagnosis and treatment of childhood-onset lupus nephritis: the SHARE initiative
Groot N, de Graeff N, Marks SD, Brogan P, Avcin T, Bader-Meunier B, Dolezalova P, Feldman BM, Kone-Paut I, Lahdenne P, McCann L, Özen S, Pilkington CA, Ravelli A, Royen-Kerkhof AV, Uziel Y, Vastert BJ, Wulffraat NM, Beresford MW, Kamphuis S.
Ann Rheum Dis. 2017 Dec;76(12):1965-73.

2017 American College of Rheumatology/American Association of Hip and Knee Surgeons Guideline for the Perioperative Management of Antirheumatic Medication in Patients With Rheumatic Diseases Undergoing Elective Total Hip or Total Knee Arthroplasty
Goodman SM, Springer B, Guyatt G, Abdel MP, Dasa V, George M, Gewurz-Singer O, Giles JT, Johnson B, Lee S, Mandl LA, Mont MA, Sculco P, Sporer S, Stryker L, Turgunbaev M, Brause B, Chen AF, Gililland J, Goodman M, Hurley-Rosenblatt A, Kirou K, Losina E, MacKenzie R, Michaud K, Mikuls T, Russell L, Sah A, Miller AS, Singh JA, Yates A.
Arthritis Rheumatol. 2017 Aug;69(8):1538-51.

EULAR recommendations for women’s health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome
Andreoli L, Bertsias GK, Agmon-Levin N, Brown S, Cervera R, Costedoat-Chalumeau N, Doria A, Fischer-Betz R, Forger F, Moraes-Fontes MF, Khamashta M, King J, Lojacono A, Marchiori F, Meroni PL, Mosca M, Motta M, Ostensen M, Pamfil C, Raio L, Schneider M, Svenungsson E, Tektonidou M, Yavuz S, Boumpas D, Tincani A.
Ann Rheum Dis. 2017 Mar;76(3):476-85.

The EULAR points to consider for use of antirheumatic drugs before pregnancy, and during pregnancy and lactation
Götestam Skorpen C, Hoeltzenbein M, Tincani A, Fischer-Betz R, Elefant E, Chambers C, da Silva J, Nelson-Piercy C, Cetin I, Costedoat-Chalumeau N, Dolhain R, Förger F, Khamashta M, Ruiz-Irastorza G, Zink A, Vencovsky J, Cutolo M, Caeyers N, Zumbühl C, Østensen M.
Ann Rheum Dis. 2016 May;75(5):795-810.

[Clinical practice guidelines for systemic lupus erythematosus: Recommendations for general clinical management]
Trujillo-Martín MM, Rúa-Figueroa Fernández de Larrinoa I, Ruíz-Irastorza G, Pego-Reigosa JM, Sabio Sánchez JM, Serrano-Aguilar P; en nombre del Grupo Elaborador de la GPC-LES.
Med Clin (Barc). 2016 May 6;146(9):413.e1-14.

[Prevention of infections in adults and adolescents with systemic lupus erythematosus: Guidelines for the clinical practice based on the literature and expert opinion]
Mathian A, Arnaud L, Adoue D, Agard C, Bader-Meunier B, Baudouin V, Belizna C, Bonnotte B, Boumedine F, Chaib A, Chauchard M, Chiche L, Daugas E, Ghali A, Gobert P, Gondran G, Guettrot-Imbert G, Hachulla E, Hamidou M, Haroche J, Hervier B, Hummel A, Jourde-Chiche N, Korganow AS, Kwon T, Le Guern V, Le Quellec A, Limal N, Magy-Bertrand N, Marianetti-Guingel P, Martin T, Martin Silva N, Meyer O, Miyara M, Morell-Dubois S, Ninet J, Pennaforte JL, Polomat K, Pourrat J, Queyrel V, Raymond I, Remy P, Sacre K, Sibilia J, Viallard JF, Viau Brabant A, Hanslik T, Amoura Z; le groupe France lupus érythémateux systémique réseau (FLEUR) et les centres de référence et de compétence des lupus et syndromes des antiphospholipides.
Rev Med Interne. 2016 May;37(5):307-20.

Clinical practice guidelines for the management of pregnancy in women with autoimmune rheumatic diseases of the Mexican College of Rheumatology. Part II
Saavedra Salinas MÁ, Barrera Cruz A, Cabral Castañeda AR, Jara Quezada LJ, Arce-Salinas CA, Álvarez Nemegyei J, Fraga Mouret A, Orozco Alcalá J, Salazar Páramo M, Cruz Reyes CV, Andrade Ortega L, Vera Lastra OL, Mendoza Pinto C, Sánchez González A, Cruz Cruz Pdel R, Morales Hernández S, Portela Hernández M, Pérez Cristóbal M, Medina García G, Hernández Romero N, Velarde Ochoa Mdel C, Navarro Zarza JE, Portillo Díaz V, Vargas Guerrero A, Goycochea Robles MV, García Figueroa JL, Barreira Mercado E, Amigo Castañeda MC.
Reumatol Clin. 2015 Sep-Oct;11(5):305-15.

Clinical practice guidelines for the management of pregnancy in women with autoimmune rheumatic diseases of the Mexican College of Rheumatology. Part I
Saavedra Salinas MÁ, Barrera Cruz A, Cabral Castañeda AR, Jara Quezada LJ, Arce-Salinas CA, Álvarez Nemegyei J, Fraga Mouret A, Orozco Alcalá J, Salazar Páramo M, Cruz Reyes CV, Andrade Ortega L, Vera Lastra OL, Mendoza Pinto C, Sánchez González A, Cruz Cruz Pdel R, Morales Hernández S, Portela Hernández M, Pérez Cristóbal M, Medina García G, Hernández Romero N, Velarde Ochoa Mdel C, Navarro Zarza JE, Portillo Díaz V, Vargas Guerrero A, Goycochea Robles MV, García Figueroa JL, Barreira Mercado E, Amigo Castañeda MC.
Reumatol Clin. 2015 Sep-Oct;11(5):295-304.

[Recommendations from the Brazilian Society of Rheumatology on the diagnosis and treatment of intestinal parasitic infections in patients with autoimmune rheumatic disorders]
Braz AS, de Andrade CA, da Mota LM, Lima CM.
Rev Bras Reumatol. 2015 Jul-Aug;55(4):368-80.

[Screening and management of cardiovascular risk factors in systemic lupus erythematosus: Recommendations for clinical practice based on the literature and expert opinion]
Arnaud L, Mathian A, Adoue D, Bader-Meunier B, Baudouin V, Belizna C, Bonnotte B, Boumedine F, Chaib A, Chauchard M, Chiche L, Daugas E, Ghali A, Gobert P, Gondran G, Guettrot-Imbert G, Hachulla E, Hamidou M, Haroche J, Hervier B, Hummel A, Jourde-Chiche N, Korganow AS, Kwon T, Le Guern V, Le Quellec A, Limal N, Magy-Bertrand N, Marianetti-Guingel P, Martin T, Martin Silva N, Meyer O, Miyara M, Morell-Dubois S, Ninet J, Papo T, Pennaforte JL, Polomat K, Pourrat J, Queyrel V, Raymond I, Remy P, Sacre K, Schmidt J, Sibilia J, Viallard JF, Viau Brabant A, Wahl D, Bruckert E, Amoura Z; groupe France Lupus Érythémateux systémique Réseau (FLEUR) et les centres de référence et de compétence des lupus et syndromes des antiphospholipides.
Rev Med Interne. 2015 Jun;36(6):372-80.

Treat-to-target in systemic lupus erythematosus: recommendations from an international task force
van Vollenhoven RF, Mosca M, Bertsias G, Isenberg D, Kuhn A, Lerstrøm K, Aringer M, Bootsma H, Boumpas D, Bruce IN, Cervera R, Clarke A, Costedoat-Chalumeau N, Czirják L, Derksen R, Dörner T, Gordon C, Graninger W, Houssiau F, Inanc M, Jacobsen S, Jayne D, Jedryka-Goral A, Levitsky A, Levy R, Mariette X, Morand E, Navarra S, Neumann I, Rahman A, Rovensky J, Smolen J, Vasconcelos C, Voskuyl A, Voss A, Zakharova H, Zoma A, Schneider M.
Ann Rheum Dis. 2014 Jun;73(6):958-67.

Joint European League Against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis
Bertsias GK, Tektonidou M, Amoura Z, Aringer M, Bajema I, Berden JH, Boletis J, Cervera R, Dörner T, Doria A, Ferrario F, Floege J, Houssiau FA, Ioannidis JP, Isenberg DA, Kallenberg CG, Lightstone L, Marks SD, Martini A, Moroni G, Neumann I, Praga M, Schneider M, Starra A, Tesar V, Vasconcelos C, van Vollenhoven RF, Zakharova H, Haubitz M, Gordon C, Jayne D, Boumpas DT; European League Against Rheumatism and European Renal Association-European Dialysis and Transplant Association.
Ann Rheum Dis. 2012 Nov;71(11):1771-82.

American College of Rheumatology guidelines for screening, treatment, and management of lupus nephritis
Hahn BH, McMahon MA, Wilkinson A, Wallace WD, Daikh DI, Fitzgerald JD, Karpouzas GA, Merrill JT, Wallace DJ, Yazdany J, Ramsey-Goldman R, Singh K, Khalighi M, Choi SI, Gogia M, Kafaja S, Kamgar M, Lau C, Martin WJ, Parikh S, Peng J, Rastogi A, Chen W, Grossman JM; American College of Rheumatology.
Arthritis Care Res (Hoboken). 2012 Jun;64(6):797-808.

EULAR recommendations for vaccination in paediatric patients with rheumatic diseases
Heijstek MW, Ott de Bruin LM, Bijl M, Borrow R, van der Klis F, Koné-Paut I, Fasth A, Minden K, Ravelli A, Abinun M, Pileggi GS, Borte M, Wulffraat NM; EULAR.
Ann Rheum Dis. 2011 Oct;70(10):1704-12.

EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations: report of a task force of the EULAR standing committee for clinical affairs
Bertsias GK, Ioannidis JP, Aringer M, Bollen E, Bombardieri S, Bruce IN, Cervera R, Dalakas M, Doria A, Hanly JG, Huizinga TW, Isenberg D, Kallenberg C, Piette JC, Schneider M, Scolding N, Smolen J, Stara A, Tassiulas I, Tektonidou M, Tincani A, van Buchem MA, van Vollenhoven R, Ward M, Gordon C, Boumpas DT.
Ann Rheum Dis. 2010 Dec;69(12):2074-82.

European League Against Rheumatism recommendations for monitoring patients with systemic lupus erythematosus in clinical practice and in observational studies
Mosca M, Tani C, Aringer M, Bombardieri S, Boumpas D, Brey R, Cervera R, Doria A, Jayne D, Khamashta MA, Kuhn A, Gordon C, Petri M, Rekvig OP, Schneider M, Sherer Y, Shoenfeld Y, Smolen JS, Talarico R, Tincani A, van Vollenhoven RF, Ward MM, Werth VP, Carmona L.
Ann Rheum Dis. 2010 Jul;69(7):1269-74.

EULAR recommendations for the management of systemic lupus erythematosus. Report of a Task Force of the EULAR Standing Committee for International Clinical Studies Including Therapeutics
Bertsias G, Ioannidis JP, Boletis J, Bombardieri S, Cervera R, Dostal C, Font J, Gilboe IM, Houssiau F, Huizinga T, Isenberg D, Kallenberg CG, Khamashta M, Piette JC, Schneider M, Smolen J, Sturfelt G, Tincani A, van Vollenhoven R, Gordon C, Boumpas DT; Task Force of the EULAR Standing Committee for International Clinical Studies Including Therapeutics.
Ann Rheum Dis. 2008 Feb;67(2):195-205.

Guidelines for immunologic laboratory testing in the rheumatic diseases: anti-Sm and anti-RNP antibody tests
Benito-Garcia E, Schur PH, Lahita R; American College of Rheumatology Ad Hoc Committee on Immunologic Testing Guidelines.
Arthritis Rheum. 2004 Dec 15;51(6):1030-44.

Guidelines for the laboratory use of autoantibody tests in the diagnosis and monitoring of autoimmune rheumatic diseases
Tozzoli R, Bizzaro N, Tonutti E, Villalta D, Bassetti D, Manoni F, Piazza A, Pradella M, Rizzotti P; Italian Society of Laboratory Medicine Study Group on the Diagnosis of Autoimmune Diseases.
Am J Clin Pathol. 2002 Feb;117(2):316-24.

Immunization consensus for children and adolescents with rheumatic diseases
Silva C, Terreri M, Barbosa C, Hilário E, Pillegi G, Ferriani V, et al.
Bras J Rheumatol. 2009 Sep-Oct;49:562-89.

What investigations are needed to optimally monitor for malignancies in SLE?
Tessier-Cloutier B, Clarke AE, Pineau CA, Keeling S, Bissonauth A, Ramsey-Goldman R, et al.
Lupus. 2015 Jul;24(8):781-7.

Optimal Monitoring For Coronary Heart Disease Risk in Patients with Systemic Lupus Erythematosus: A Systematic Review
Tselios K, Sheane BJ, Gladman DD, Urowitz MB.
J Rheumatol. 2016 Jan;43(1):54-65.

Optimal management of fatigue in patients with systemic lupus erythematosus: a systematic review
Yuen HK, Cunningham MA.
Ther Clin Risk Manag. 2014 Oct;10:775-86.

SLE centres in ERN ReCONNET

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