Management of systemic lupus erythematosus (SLE)

Management for systemic lupus erythematosus (SLE) can be divided into pharmacological and non pharmacological. For non pharmacological, it will involve more on counseling and social support. SLE is a systemic disease that involve with a lot of stress, more understanding of the disease and its manifestation will improve the acceptance of the patient and will enhance the compliance to the treatment. It will help patient to prevent trigger factors and to monitor their illness. Counseling should also be given to family members and friends to ensure strong social support.

Pharmacological treatment for SLE is mainly depending on the type and severity of disease manifestations. The main goal of treatment is to control the acute and severe flares and to develop maintenance strategies where symptoms are suppressed to an acceptable level where the patients can continue with his/her daily activities without much hurdle (1).

There are few factors that will help to determine the treatment of choice. First of all it is need to be decided either the disease is life threatening or not or is there any target organ damage. The other thing that should be taken into consideration is whether manifestations of the disease itself are reversible or not. The management should be the best approach to prevent complication of the disease and treatment.

For non life threatening disease, conservative therapy can be applied. NSAIDs can be given for the management of pain (for example tablet ibuprofen 400-800mg 3 or 4 times daily. Antimalarials (e.g.: hydroxychloroquine) can be given to improve some constitutional and cutaneous symptoms. As addition, it can relieve articular manifestation as well. Usually, tablet hydroxychloroquine 400mg daily will be given. Hydroxychloroquine is a successful drug for control some manifestations of lupus, especially skin and joint disease, but those taking hydroxychloroquine for more than 7 years or who are at increased risk may need more frequent monitoring (2). Furthermore, before and after initiation of treatment, ophtalmologic evaluation is necessary to rule out ocular toxicity (1).

For life threatening SLE, systemic glucocorticoid is indicated (1). A study has shown that corticosteroid is a risk factor for cardiovascular disease among SLE patients and the patients also have higher risk for hypertension and dyslipidaemia (3). Therefore, it is possible that corticosteroid might be associated with rapid aging of arteries (3).

Cytotoxic agents play a rule in active glomerulonephritis. It should be considered in severe disease that cannot be controlled by steroid. Cyclophosphamide is considered to be a standard drug to start. It can control the life threatening active lupus nephritis. Recommended dosage is 7-25mg/kg every 4 weeks and should be administered intravenously. Daily oral dosing is between 1.5-2.5 mg/kg per day. Caution should be taken because oral drug carries higher risk of urinary bladder toxicity. Short term studies has been done on mycophenolate mofetil suggest efficacy in some patients. If the patient is contraindicated to unable to tolerate cyclophosphamide, azathioprine can be given as alternative (1).  Adding cyclophosphamide or azathioprine to steroids therapy gives a better outcome compare to steroids alone. Combination of these drugs has improves the kidney functions, surprisingly it won’t reduce kidney failure. Moreover, it is important to take a note that cyclophosphamide may itself cause infertility (4).

Anticoagulation is indicated in patients with thrombotic complications. Usually long term warfarin will be given to the patient. The INR need to be keep between 2.5 to 3.5 . If the patients are expecting conception or is currently pregnant, warfarin should not be used to the patients between weeks 6 to 12 weeks of pregnancy due to risk of embroyopathy (5). During this time, subcutaneous clexane will be given to give protection against thromboembolic disease. However, many doctors try to play safe and practice not to give warfarin during pregnancy to SLE patients.

For the management of autoimmune hemolytic anemia, blood transfusion is needed if the patient severely anemic and symptomatic. Glucocorticoids will be prescribed. In some cases, the patient may need immunosuppressive agents, danazol, plasmapheresis and rituximab (1). B-cell-depletion therapy with rituximab is efficacious and safe to treat AIHA and autoimmune thrombocytopenia particularly in pediatrics patients with SLE (6).

 

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Reference:

  1. Kasper DL, Braunwald E, Fauci AS, Hauser SL,  Longo DL, Jameson JL. Harrison’s principle of internal medicine. Manual of medicine, 16th edition, International edition, editors.Mc Graw Hill.2005
  2. Coleman SR, Curier RV, Katz JSD.  Primary care management of SLE. Patient Care July 2003;37.
  3. Amir T, Eyal, E, Angela, F, Reuven, Z, Dov, G. Vascular Elasticity of Systemic Lupus Erythematosus Patients Is Associated with Steroids and Hydroxychloroquine Treatment.Annals of the New York Academy of Sciences.2007;1108(1):24-34.
  4. Flanc RS, Roberts MA, Strippoli GFM, Chadban SJ, Kerr PG, Atkins RC. Treatment for lupus nephritis. Cochrane Database of Systematic Reviews 2004, Issue 1.6 pages
  5. Guillermo RI, Munther, KA. Management of Thrombosis in Antiphospholipid Syndrome and Systemic Lupus Erythematosus in Pregnancy.Annals of the New York Academy of Sciences. 2005; 1051:606-612.
  6. Kumar S, Benseler SM, Kirby-Allen M, Silverman ED. B-Cell Depletion for Autoimmune Thrombocytopenia and Autoimmune Hemolytic Anemia in Pediatric Systemic Lupus Erythematosus. Pediatrics. 2009; 123 (1): 159-163.

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