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SAMJ: South African Medical Journal

On-line version ISSN 2078-5135
Print version ISSN 0256-9574

SAMJ, S. Afr. med. j. vol.106 n.3 Pretoria Mar. 2016


8. Bertsias GK, Tektonidou M, Amoura Z, et al. 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. Ann Rheum Dis 2012;71:1771-1782. []        [ Links ]

9. Chan TM. Treatment of severe lupus nephritis: The new horizon. Nat Rev Nephrol 2015;11:46-61. []        [ Links ]

10. Day CJ, Lipkin GW, Savage CO. Lupus nephritis and pregnancy in the 21st century. Nephrol Dial Transplant 2009;24:344-347. []        [ Links ]

11. Gordon C, Jayne D, Pusey C, et al. European consensus statement on the terminology used in the management of lupus glomerulonephritis. Lupus 2009;18:257-263. []        [ Links ]



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Evidence-based treatment of systemic lupus erythematosus and its complications



B HodkinsonI, II; M A MakdaIII

IMB BCh, PhD, FCP (SA), Cert Rheumatology (SA).Department of Medicine, Faculty of Health Sciences, Groote Schuur Hospital and University of Cape Town, South Africa
IIMB BCh, PhD, FCP (SA), Cert Rheumatology (SA). Division of Rheumatology, Department of Internal Medicine, Chris Hani Baragwanath Academic Hospital, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa
IIIMB BCh, FCP (SA), Cert Rheumatology (SA), MMed (Int Med). Division of Rheumatology, Department of Internal Medicine, Chris Hani Baragwanath Academic Hospital, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa





Outcomes for patients with systemic lupus erythematosus (SLE) have improved during the last two decades as our understanding of the disease expands. In particular, the importance of antimalarial therapy for addressing and preventing a host of complications in SLE has emerged. Furthermore, evidence is mounting that corticosteroids, while offering excellent control of disease activity, are responsible for many of the late complications of SLE and need to be prescribed in modest doses for the shortest time possible. To achieve this, an understanding of the available 'steroid-sparing' immunosuppressants is useful. Specific attention needs to be paid to the two most important complications of SLE, i.e. infections and atherosclerotic cardiovascular events. Awareness of, screening for and aggressive management of risk factors for these comorbidities are paramount.



Rationale for this review

Ideally, all systemic lupus erythematosus (SLE) patients should be reviewed by a specialist. Early referral to a rheumatologist has been associated with faster treatment modification and better outcomes. However, primary care doctors are the first point of care for new SLE patients or those presenting with a new complication. Moreover, given the shortage of rheumatologists in South Africa (SA) and elsewhere in Africa, primary care clinicians and general physicians are often faced with sick SLE patients and are required to make rapid therapeutic decisions. This review summarises current evidence for the treatment of SLE and its complications. We prepared this review after a PubMed search using the words 'systemic lupus erythematosus' and 'therapy'.


General principles of management

Education and support

At diagnosis, patients should be offered counselling and educational material, preferably by a rheumatology nurse. Lupus support groups are very beneficial. Patients should avoid sun exposure and use a daily sunscreen (sun protection factor (SPF) >50).

Antimalarial therapy

The antimalarial (AM) drugs chloroquine (CQ) and hydroxychloroquine (HQ) have been used in the treatment of SLE for >50 years, with CQ being much more readily available in SA than HQ. Recent studies demonstrate their anti-inflammatory, antithrombotic and antilipidaemic effects via multiple molecular pathways, resulting in better control of disease activity and fewer complications (Table 1).[1] Given the current evidence, all SLE patients should be prescribed an AM, and clinicians should encourage compliance. The common side-effects of AM drugs are gastrointestinal, but these are usually mild and transient. More worrisome are skin hyperpigmentation and maculopathy. The latter is uncommon, affecting 2.5% of patients treated with CQ for 10 years (and 0.1% of patients using HQ); the major risk factor seems to be the cumulative dose of an AM.[2] If diagnosed early, and the AM is discontinued, the maculopathy is reversible. Therefore, patients on AMs should undergo ophthalmological assessment including fundoscopy, visual field tests and optical coherence tomography at baseline and annually after 5 years of CQ use.



Corticosteroids - less is more

Corticosteroids (CS) offer excellent control of active disease, but are responsible for most of the irreversible organ damage in SLE patients 15 years after diagnosis.[3] The damage is proportional to both the cumulative dose of CS and the average daily dose, and even low doses (<7.5 mg daily) are associated with a risk of cataracts, cardiovascular (CV) disease and osteoporosis.[4] The best practice is to prescribe CS only to patients with severe organ involvement at the lowest effective dose for the shortest duration of time possible. A recent study demonstrated that moderate doses (<30 mg/day) of CS for severe non-renal SLE were as effective and associated with fewer adverse effects than high doses.[5] Another study demonstrated that after pulse methylprednisolone, oral CS may be omitted from an induction immunosuppressive regimen for nephritis.[6] An understanding of currently available immunosuppressant drugs such as methotrexate (MTX), azathioprine (AZA), cyclophosphamide (CYC) and mycophenylate mofetil (MMF) is vital in a steroid-sparing approach (Table 2).

Monitoring SLE patients

For patients with inactive disease, 4-monthly visits should include a clinical examination, blood pressure measurement, urine dipstick test, full blood count, and liver transaminase for those using potentially hepatotoxic medication. Other serum markers of active disease such as complement (C3 and C4), double-stranded DNA (dsDNA), and C-reactive protein may be useful for follow-up, but do not accurately predict flares. An annual lipogram, creatinine measurement, and HIV test are recommended. Screening for antiphospholipid antibodies at diagnosis is useful. As therapy of acute SLE improves, patients live longer. Therefore, comorbidities of the disease or complications of therapy have emerged as important causes of morbidity and mortality. Preventive and therapeutic strategies are summarised in Table 3.


The combination of inherent immune dysfunction and immuno-suppressant therapy, in particular CS, puts SLE patients at high risk of community-acquired and opportunistic infections.[7] These infections contribute significantly to the hospitalisation and mortality of SLE patients, and need to be distinguished from an SLE flare, bearing in mind that infections may also occur together with flares. Vaccination, vigilance, easy access to well-informed clinicians and prompt antibiotic therapy are important measures. Moreover, SLE patients are at higher risk of pulmonary and extrapulmonary tuberculosis (TB); this risk is related to CS use, lymphopenia, and lupus disease itself.[8] Clinicians need to consider the possibility of TB in any ill SLE patient.


Patients with SLE have a 2 - 5-fold increased risk of cardiac, cerebral or peripheral art erial atherosclerosic events, and CV events are the leading cause of mortality in SLE.[9] Traditional Framingham CV risk factors do not fully explain the extent of the risk, as inflammatory and prothrombotic mechanisms underlie this premature atherosclerosis. Encouragingly, the CV risk may be reduced by regular exercise, statin therapy and possibly low-dose aspirin for any patient with >1 traditional risk factors for atherosclerotic disease, or with antiphospholipid antibodies.[10,11]

Antiphospholipid syndrome

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