- The British Society of Rheumatology has published guidelines for the treatment of systemic lupus erythematosus (SLE), an autoimmune condition
- The treatment differs for mild, moderate and severe cases
- Corticosteroids should be kept at a minimum with hydroxychloroquine being the preferred drug for long-term maintenance.
Recent guidelines published by the British Society for Rheumatology will help rheumatologists and other health care professionals working with patients with systemic lupus erythematosus (SLE)in the United Kingdom to treat them appropriately.
Systemic lupus erythematosus (SLE) is an autoimmune disease that causes skin rash and can affect multiple organs including the kidneys, joints and the brain. The disease was in the news recently when the singer and actress Selena Gomez underwent a kidney transplant following failure of her kidneys due to SLE.
The diagnosis of SLE is based on clinical features and is confirmed by the presence of autoantibodies like antinuclear antibody (ANA) which is positive in around 95% cases, anti-dsDNA antibodies, and anti-Smith (Sm) antibodies, and low complement levels.
The treatment of SLE depends on its severity – SLE may be mild, moderate or severe, or may occur as flares. The following is a summary of the guidelines issued by the Royal Society of Rheumatology for its treatment.
Mild SLE usually involves the skin and mucous membranes, joints or the pleura (tissue covering the lungs), but is not associated with life-threatening organ involvement. The symptoms may include fatigue, rash on the cheeks, diffuse hair loss, mouth ulcers, joint or muscle pain, and a platelet count of between 50 and 149 × 109/l.
Drugs that can be used for the treatment of mild SLE include:
- Initial treatment can be with local corticosteroid, oral prednisolone ≤20 mg daily for 1-2 weeks, or intramuscular or intraarticular (injection into the joint) injection of methyl-prednisolone in a dose ranging from 80 to120 mg.
- In addition, the disease-modifying drugs hydroxychloroquine and /or methotrexate and/or short courses of NSAIDs (non-steroidal painkillers like ibuprofen and diclofenac) may be used.
- Low dose prednisolone (less than or equal to 7.5 mg/day), hydroxychloroquine and/or methotrexate may be used for maintenance treatment. Corticosteroids may be used locally on the skin or can be injected into the joints for arthritis.
- High-Sun Protection Factor (SPF) sunscreen along with avoiding sunlight is advised to prevent skin lesions.
Moderate SLE is characterized by fever, rash up to 2/9 body surface area, cutaneous vasculitis (inflammation of the blood vessels of the skin), hair loss with scalp inflammation, liver or joint disease, inflammation of the pleura or pericardium (the tissues surrounding the lungs and the heart, respectively) and a platelet count of 25-49 × 109/l. Moderate SLE requires the following drugs:
- Corticosteroids in the form of higher doses of prednisolone (up to 0.5 mg/kg/day), or the use of intramuscular or intravenous injections of methylprednisolone.
- Methotrexate, azathioprine, mycophenolate mofetil, ciclosporin or other calcineurin inhibitors may be used in addition to hydroxychloroquine for patients with skin or joint disease, vasculitis or low blood counts.
- For cases that do not respond to the above treatments, belimumab or rituximab may be tried out.
- Maintenance treatment is with prednisolone, hydroxychloroquine, and azathioprine, methotrexate, mycophenolate mofetil or cyclosporine.
Severe SLE is a life-threatening condition that can affect vital organs including the kidneys and the brain. The patient may present with rash involving more than 2/9 body surface area, muscle pain, severe inflammation of the pleura and/or pericardium with fluid accumulation, fluid accumulation in the abdomen, involvement of the intestines, brain, spinal cord, and optic nerve, and an extremely low platelet count of less than 25 × 109/l. The presence of infection should be ruled out in these patients.
- Intravenous methylprednisolone or high-dose oral prednisolone (up to 1 mg/kg/day) may be required as initial treatment.
- Immunosuppressive drugs that may be used include mycophenolate mofetil and ciclosporin for lupus nephritis and refractory severe non-kidney disease in addition to the corticosteroid treatment in addition to hydroxychloroquine.
- The biologic therapies belimumab or rituximab may be used for patients who have failed to respond to the immunosuppressive drugs or cannot use them due to side effects.
- Intravenous immunoglobulin, plasmapheresis (removal of antibodies from the plasma) may be required for patients with low blood counts not responding to treatment, thrombotic thrombocytopenic purpura (due to low platelet levels), rapidly deteriorating acute confusional state and the catastrophic variant of APS (antiphospholipid syndrome).
- Anticoagulation may be needed for patients with thrombotic manifestations
- Maintenance treatment is with prednisolone and azathioprine, mycophenolate mofetil or cyclosporine, along with hydroxychloroquine.
The treatment for flares depends on the severity of the underlying disease. Most drugs except hydroxychloroquine can be eventually stopped once the patient achieves stable remission.
- Gordon C et al. The British Society for Rheumatology guideline for the management of systemic lupus erythematosus in adults: Executive Summary. Rheumatology, Volume 57, Issue 1, 1 January 2018, Pages 14-18, https:doi.org/10.1093/rheumatology/kex291