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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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