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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.

Disease_SLE
Disease_SLE

LIST OF USEFUL REFERENCES

1. 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, et al.
Ann Rheum Dis. 2017 Mar;76(3):476-85.

2. [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, et al.
Rev Med Interne. 2015 Jun;36(6):372-80.

3. 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 G.
Arthritis Rheum. 2004 Dec 15;51(6):1030-44.

4. 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, et al.
Ann Rheum Dis. 2008 Feb;67(2):195-205.

5. 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, et al.
Ann Rheum Dis. 2010 Dec;69(12):2074-82.

6. 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, et al.
Ann Rheum Dis. 2012 Nov;71(11):1771-82.

7. [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.

8. 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, et al.
Arthritis Rheumatol. 2017 Aug;69(8):1538-51.

9. 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, et al.
Ann Rheum Dis. 2017 Dec;76(12):1965-73.

10. 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, et al.
Arthritis Care Res (Hoboken). 2012 Jun;64(6):797-808

11. EULAR recommendations for vaccination in paediatric patients with rheumatic diseases
Heijstek MW, Ott de Bruin LM, Bijl M, Borrow R, van der Klis F, Kone-Paut I, et al.
Ann Rheum Dis. 2011 Oct;70(10):1704-12.

12. [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, et al.
Rev Med Interne. 2016 May;37(5):307-20.

13. 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, et al.
Ann Rheum Dis. 2010 Jul;69(7):1269-74.

14. Clinical practice guidelines for the management of pregnancy in women with autoimmune rheumatic diseases of the Mexican College of Rheumatology. Part I
Saavedra Salinas MA, Barrera Cruz A, Cabral Castaneda AR, Jara Quezada LJ, Arce-Salinas CA, Alvarez Nemegyei J, et al.
Reumatol Clin. 2015 Sep-Oct;11(5):295-304.

15. Clinical practice guidelines for the management of pregnancy in women with autoimmune rheumatic diseases of the Mexican College of Rheumatology. Part II
Saavedra Salinas MA, Barrera Cruz A, Cabral Castaneda AR, Jara Quezada LJ, Arce-Salinas CA, Alvarez Nemegyei J, et al.
Reumatol Clin. 2015 Sep-Oct;11(5):305-15.

16. 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.

17. 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.

18. 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, et al.
Am J Clin Pathol. 2002 Feb;117(2):316-24.

19. [Clinical practice guidelines for systemic lupus erythematosus: Recommendations for general clinical management]
Trujillo-Martin MM, Rua-Figueroa Fernandez de Larrinoa I, Ruiz-Irastorza G, Pego-Reigosa JM, Sabio Sanchez JM, Serrano-Aguilar P, et al.
Med Clin (Barc). 2016 May;146(9):413 e1-14.

20. 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.

21. Treat-to-target in systemic lupus erythematosus: recommendations from an international task force
van Vollenhoven RF, Mosca M, Bertsias G, Isenberg D, Kuhn A, Lerstrom K, et al.
Ann Rheum Dis. 2014 Jun;73(6):958-67.

22. 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.

23. 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, Fˆrger 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.

24. 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.

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