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Management of Systemic Lupus Erythematosus

• minimal prednisolone dose of ≤7.5 mg/day
• on standard maintenance doses of immunosuppressive drugs
and/or approved biological agents
It is estimated that 20 - 25% of SLE patients will flare within 1 - 2 years
and 40 - 66% within 5 - 10 years after achievement of a low disease
activity or remission. The risk factors for SLE flare include: 43, level III
• male gender
• age of SLE onset ≤25 years
• major organ involvement (cytopenias, neuropsychiatric, nephritis,
vasculitis)
• persistent clinical disease activity
• low serum C3/C4
• high anti-dsDNA antibodies
• poor compliance to treatment
• discontinuation or have never been on HCQ
• rapid tapering or withdrawal of maintenance immunosuppressive
treatment

Smoking and ultraviolet radiation have been shown to
trigger or aggravate cutaneous lupus erythematosus (CLE)
manifestations. 44 - 45, level III There is a small increase in risk of mild to
moderate lupus flares with use of oral HRT. 46
In a meta-analysis of moderate quality primary studies on the effect
of disease activity and damage of patients with SLE, it was found that
there were: 47, level I
• greater risk of mortality in patients with higher SLEDAI disease
activity (HR=1.14, 95% CI 1.06 to 1.22)
• greater risk of damage in patients with higher SLEDAI scores as
measured by SLICC/ACR Damage Index (SDI) (HR=1.18, 95% CI
1.02 to 1.37)
• higher risk of mortality in patients with worse damage as measured
by SDI (HR=1.44, 95% CI 1.29 to 1.61)
The above findings were supported by a large meta-analysis that
showed organ damage with greater SDI in SLE was consistently
associated with increased mortality (HR=1.34, 95% CI 1.24 to 1.44).
Most of the primary studies were of high-quality. 48, level II-2

There is no standard definition of refractory disease in SLE. The
CPG DG opines that it may be defined as persistent disease activity
despite optimal standard therapy. However, it is important to ensure the
diagnosis is accurate and, persistent symptoms and signs are derived
primarily from SLE activity and not from concomitant diseases (e.g.
infections, atherosclerosis or other autoimmune diseases) or adverse
events (AEs) of drugs. 49, level III

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