{"id":1090,"date":"2023-12-11T17:19:58","date_gmt":"2023-12-11T17:19:58","guid":{"rendered":"https:\/\/blogs.bmj.com\/rheumsummaries\/?p=1090"},"modified":"2023-12-11T17:22:37","modified_gmt":"2023-12-11T17:22:37","slug":"updated-sle-recommendations","status":"publish","type":"post","link":"https:\/\/blogs.bmj.com\/rheumsummaries\/2023\/12\/11\/updated-sle-recommendations\/","title":{"rendered":"Updated: SLE recommendations"},"content":{"rendered":"<p><span style=\"font-weight: 400\">This is the lay version of the EULAR recommendations for managing systemic lupus erythematosus. The\u00a0 original publication can be downloaded from the EULAR website: www.eular.org.\u00a0<\/span><\/p>\n<p><a href=\"https:\/\/ard.bmj.com\/content\/early\/2023\/10\/11\/ard-2023-224762\"><span style=\"font-weight: 400\">Fanouriakis A, Kostopoulou M, Andersen J, et al EULAR recommendations for the management of systemic <\/span><span style=\"font-weight: 400\">\u00a0<\/span><span style=\"font-weight: 400\">lupus erythematosus: 2023 update Annals of the Rheumatic Diseases Published Online First: 12 October <\/span><span style=\"font-weight: 400\">\u00a0<\/span><span style=\"font-weight: 400\">2023. doi: 10.1136\/ard-2023-224762<\/span><span style=\"font-weight: 400\">\u00a0<\/span><\/a><\/p>\n<p><b>Introduction<br \/>\n<\/b><span style=\"font-weight: 400\">EULAR \u2013 the European Alliance of Associations for Rheumatology \u2013 gives advice to doctors, nurses, and\u00a0 patients about the best way to treat and manage diseases. In 2023, EULAR updated its recommendations on\u00a0 systemic lupus erythematosus (often shortened to SLE). Doctors, nurses, other health professionals, and\u00a0 patients worked together to develop this new advice. The patients in the team ensured that the patient point\u00a0 of view was included.\u00a0\u00a0<\/span><\/p>\n<p><b>What do we already know?<br \/>\n<\/b><span style=\"font-weight: 400\">SLE is an inflammatory autoimmune disease, where the immune system mistakenly attacks tissues in the\u00a0 body. SLE does not look the same in every patient, but it can affect all organs or tissues in the body, including\u00a0 the skin, joints, kidneys, nervous system, lungs, heart, and blood cells. People with SLE can have different\u00a0 symptoms, including fatigue, rash, and joint pain or swelling. There are also a number of different antibodies\u00a0 associated with SLE, which might be detected in a blood test. These include antinuclear antibodies (ANA) and\u00a0 antiphospholipid antibodies (aPL), among others.\u00a0\u00a0<\/span><\/p>\n<p><span style=\"font-weight: 400\">EULAR first published recommendations around the management of SLE in 2008. Traditionally, there have\u00a0 not been many treatment options for this disease. However, since the last update in 2019, new drugs have\u00a0 been approved and options are beginning to expand.\u00a0\u00a0<\/span><\/p>\n<p><span style=\"font-weight: 400\">Of note, the previous recommendations included 33 statements across four topics. This update has only 13 statements. This structural change is in line with current EULAR practice to streamline the number of\u00a0 recommendation statements in any one publication. Because of this change, all overarching principles and\u00a0 individual recommendations are considered to be new, even where the essence has remained unchanged from the previous version.\u00a0\u00a0<\/span><\/p>\n<p><b>What do the recommendations say?<br \/>\n<\/b><span style=\"font-weight: 400\">In total, there are 5 overarching principles and 13 recommendations. The principles say that people with SLE\u00a0 require multidisciplinary, individualized management based on patient education and shared decision-making \u2013 and taking into consideration costs to both the patient and society. Disease activity should be assessed at\u00a0 each clinic visit, and organ damage checked at least annually. Non-pharmacological interventions, such as\u00a0 using sun protection, exercising, stopping smoking, and eating a healthy diet, as well as measures to promote\u00a0 bone health, are important to improve long-term outcomes. Drug treatments should be chosen based on each\u00a0 person\u2019s individual characteristics and preferences, as well as their disease type and severity of organ\u00a0 involvement. Early diagnosis, regular organ screening, prompt treatment, and strict adherence are essential\u00a0 to prevent flares and organ damage, as well as to improve the long-term outcome, and enhance quality of life.\u00a0<\/span><\/p>\n<p><span style=\"font-weight: 400\">Each recommendation is based on the best current knowledge from studies of scientific evidence or expert\u00a0 opinion. The more stars a recommendation has the stronger the evidence is. However, recommendations with limited scientific evidence may be important, because the experts can have a strong opinion even when\u00a0 the published evidence may be lacking.\u00a0<\/span><\/p>\n<p><span style=\"font-weight: 400\">One star (*) means it is a recommendation with limited scientific evidence.<br \/>\n<\/span><span style=\"font-weight: 400\">Two stars (**) means it is a recommendation with some scientific evidence.<br \/>\n<\/span><span style=\"font-weight: 400\">Three stars (***) means it is a recommendation with quite a lot of scientific evidence.<br \/>\n<\/span><span style=\"font-weight: 400\">Four stars (****) means it is a recommendation supported with a lot of scientific evidence.<\/span><span style=\"font-weight: 400\">\u00a0<\/span><\/p>\n<p><b>Recommendations\u00a0<\/b><\/p>\n<ul>\n<li><b>Hydroxychloroquine is the mainstay of treatment for SLE.***<br \/>\n<\/b>An antimalarial drug called hydroxychloroquine is recommended for everyone with SLE, unless there is\u00a0 a specific reason why you cannot take it (a contraindication). The target dose is based on your weight,\u00a0 and is 5 mg\/kg per day. However, this advice should be tailored based on each person\u2019s individual risk\u00a0 for flare and eye toxicity.<\/li>\n<\/ul>\n<ul>\n<li><b>If you need glucocorticoids, the dose will depend on your organ involvement, but should be low\u00a0 and temporary. Pulses of intravenous methylprednisolone can be considered in some people.**<br \/>\n<\/b>Glucocorticoids are a type of steroid drugs. The dose should be based on the type and severity of your\u00a0 organ involvement. The dose should be reduced to maintenance dose of \u22645 mg\/day (prednisone\u00a0 equivalent) and, when possible, withdrawn, because long-term therapy with glucocorticoids is associated\u00a0 with adverse effects. If you have moderate-to-severe disease, pulses of intravenous methylprednisolone\u00a0 (125\u20131000 mg\/day for 1\u20133 days) can be considered.<\/li>\n<\/ul>\n<ul>\n<li><b>If you do not respond to hydroxychloroquine or are unable to reduce your steroid dose,\u00a0 immunosuppressive*** and\/or biologic agents**** should be considered.<br \/>\n<\/b>Some people will not respond to hydroxychloroquine, alone or in combination with glucocorticoids.\u00a0 Others might not be able to reduce their glucocorticoid doses low enough for it to be used for a long\u00a0 period. If this is the case, other drugs should be added. Your healthcare team might consider adding immunomodulating or immunosuppressive agents such as methotrexate, azathioprine, or\u00a0 mycophenolate, or they might consider adding belimumab or anifrolumab.<\/li>\n<\/ul>\n<ul>\n<li><b>In people with organ- or life-threatening disease, intravenous cyclophosphamide should be\u00a0 considered; in refractory cases, rituximab may be considered.**<br \/>\n<\/b>Some people with SLE will have serious organ- or life-threatening disease that might need treatment\u00a0 with intravenous cyclophosphamide. Rituximab is also an option for people with refractory disease.<\/li>\n<\/ul>\n<ul>\n<li><b>If you have active skin disease, initial treatment should include topical agents,*** antimalarials,**** and\/or glucocorticoids.** Immunosuppressives or biologics can be considered\u00a0 as second-line.<br \/>\n<\/b>The first-line treatment for active skin disease is topical glucocorticoids or calcineurin inhibitors,\u00a0 antimalarials (hydroxychloroquine, chloroquine), and\/or systemic glucocorticoids. Around 40% of people\u00a0 will not respond to first-line treatments, and they can then try methotrexate,*** mycophenolate,** anifrolumab,**** or belimumab*** as second-line therapies.<\/li>\n<\/ul>\n<ul>\n<li><b>If you have active neuropsychiatric disease caused by SLE, you might be offered\u00a0 glucocorticoids and immunosuppressives,**** or antiplatelet agents\/anticoagulants** \u2013 this will\u00a0 depend on your specific neuropsychiatric symptom(s).<br \/>\n<\/b>Some people with SLE will have neuropsychiatric symptoms. It can be difficult to link any symptoms to\u00a0 your SLE. Depending on your specific problems, you might be offered glucocorticoids and\u00a0 immunosuppressives agents for inflammatory manifestations such as myelopathy or an acute\u00a0 confusional state. Or you might be given antiplatelet agents or anticoagulants if you have\u00a0 cerebrovascular disease or a stroke.<\/li>\n<\/ul>\n<ul>\n<li><b>If you have acute severe autoimmune thrombocytopenia, you might need high-dose glucocorticoids*** with or without immunoglobulin G,** rituximab,*** and cyclophosphamide** followed by maintenance therapy.<br \/>\n<\/b>If you have severe autoimmune thrombocytopenia, you might be treated with high-dose glucocorticoids\u00a0 (including pulses of intravenous methylprednisolone) \u2013 with or without intravenous immunoglobulin G,\u00a0 rituximab, or high-dose intravenous cyclophosphamide. This initial treatment might be followed by\u00a0 maintenance with rituximab, azathioprine, mycophenolate, or cyclosporine.<\/li>\n<\/ul>\n<ul>\n<li><b>People with active proliferative lupus nephritis should receive low-dose cyclophosphamide or\u00a0 mycophenolate and glucocorticoids. Combination therapy should be considered.****<br \/>\n<\/b>Lupus nephritis is the term used when the disease affects the kidneys. People with active proliferative\u00a0 lupus nephritis should receive low-dose intravenous cyclophosphamide or mycophenolate and pulses of\u00a0 intravenous methylprednisolone followed by lower oral doses. Your healthcare team may also consider\u00a0 using combination therapy, for example, belimumab plus cyclophosphamide or mycophenolate, or\u00a0 calcineurin inhibitors such as voclosporin or tacrolimus plus mycophenolate.<\/li>\n<\/ul>\n<ul>\n<li><b>After renal response, lupus nephritis treatment should continue for at least 3 years.***<br \/>\n<\/b>If your kidneys respond to therapy, your lupus nephritis treatment should continue for at least 3 years. If\u00a0 you were initially treated with mycophenolate alone or in combination with belimumab or a calcineurin\u00a0 inhibitor, you should remain on these drugs. If you were initially treated with cyclophosphamide \u2013 either\u00a0 on its own or in combination with belimumab \u2013 then cyclophosphamide should be replaced with\u00a0 azathioprine or mycophenolate.<\/li>\n<\/ul>\n<ul>\n<li><b>In you are at high-risk for renal failure, you may receive high-dose cyclophosphamide in\u00a0 combination with pulse methylprednisolone.****<br \/>\n<\/b>Being at high risk of renal (kidney) failure is defined as having a reduced function called GFR (glomerular filtration rate), presence in your kidney biosy specimen of cellular crescents or fibrinoid\u00a0 necrosis, or severe interstitial inflammation, at the onset of nephritis. If this is your case, your healthcare\u00a0 team may consider using high-dose intravenous cyclophosphamide in combination with pulse intravenous methylprednisolone.<\/li>\n<\/ul>\n<ul>\n<li><b>If you achieve sustained remission, you might be able to gradually reduce your treatment.\u00a0 Glucocorticoids should be stopped first.***<br \/>\n<\/b>Remission is when you have no signs or symptoms of your disease. If this happens and is sustained for\u00a0 a period of time, you may be able to gradually taper (reduce) your treatment. Any glucocorticoids should\u00a0 be withdrawn first, before trying to taper any other treatments.<\/li>\n<\/ul>\n<ul>\n<li><b>If you have thrombotic antiphospholipid syndrome and have a blood clot, you will need long term vitamin K antagonists. Low dose aspirin should also be considered in people with a high risk antiphospholipid antibody profile.***<br \/>\n<\/b>SLE associated with thrombotic antiphospholipid syndrome (APS) should be managed with long-term\u00a0 vitamin K antagonists, such as warfarin, after the first arterial or unprovoked venous thrombotic event (blood clot). Low-dose aspirin (75\u2013100 mg\/day) should be considered in people with SLE without a\u00a0 thrombotic event, but who have a high-risk antiphospholipid antibody profile.<\/li>\n<\/ul>\n<ul>\n<li><b>You can take care of your health by keeping up-to-date with vaccinations, managing bone\u00a0 health, kidney and cardiovascular risks, and attending screening for malignancies.*<br \/>\n<\/b>People with rheumatic diseases such as SLE can be at a higher risk of infections than other people.\u00a0 Vaccines (immunizations) can help prevent infections. You should consider getting vaccinated against\u00a0 herpes zoster virus, human papillomavirus, influenza, COVID-19, and pneumococcus. You can also <span style=\"font-size: 1rem\">take steps to manage the health of your bones, kidneys, and heart. Screening for malignancies should\u00a0 also be performed.\u00a0\u00a0<\/span><\/li>\n<\/ul>\n<p><b>Summary<br \/>\n<\/b><span style=\"font-weight: 400\">Overall, these recommendations give guidance to health professionals and patients about the management\u00a0 and treatment of people with SLE. You should work with your healthcare team to make an informed decision\u00a0 about your disease and its treatment. These recommendations focus on pharmacological options, but there\u00a0 are also non-pharmacological treatments that might help your disease. Separate recommendations have been\u00a0 published, and you can read them <\/span><span style=\"font-weight: 400\">here<\/span><span style=\"font-weight: 400\">.\u00a0\u00a0<\/span><\/p>\n<p><span style=\"font-weight: 400\">Recommendations with just one or two stars are based mainly on expert opinion and not backed up by studies,\u00a0 but these may be as important as those with three or four stars.\u00a0<\/span><\/p>\n<p><span style=\"font-weight: 400\">If you have any questions or concerns about your disease or your medication, you should speak to a health\u00a0 professional involved in your care.\u00a0<\/span><\/p>\n","protected":false},"excerpt":{"rendered":"<p>This is the lay version of the EULAR recommendations for managing systemic lupus erythematosus. The\u00a0 original publication can be downloaded from the EULAR website: www.eular.org.\u00a0 Fanouriakis A, Kostopoulou M, Andersen J, et al EULAR recommendations for the management of systemic \u00a0lupus erythematosus: 2023 update Annals of the Rheumatic Diseases Published Online First: 12 October \u00a02023. 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