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Management of Systemic Lupus Erythematosus

           that is no worse than 20% below the pre-flare value or ≤60 mL/min/
           1.73 m  and no rescue therapy for treatment failure) at week 104. 85, level I
                 2
           8.2  Mucocutaneous

           Topical  agents  (corticosteroids  or  CNIs)  and  HCQ  are  the  mainstay
           of  treatment  in  CLE.  Systemic  corticosteroids  may  be  considered  in
           moderate to severe CLE or when topical treatment is insufficient or not
           practical. 23
           In a Cochrane systematic review of 61 RCTs with interventions for CLE
           showed: 86, level I
             •  HCQ was more effective than placebo in achieving partial clinical
               response at 12 months although the difference was not significant
             •  HCQ  was  superior  to  placebo  on  reducing  clinical  flares  at  six
               months (RR=0.49, 95% CI 0.28 to 0.89)
             •  MTX  was  superior  to  placebo  in  achieving  complete  clinical
               response at six months (RR=3.57, 95% CI 1.63 to 7.84)
             •  MTX  led  to  fewer  flares  compared  with  placebo  at  12  months,
               however it was not significant
             •  no difference between AZA  and ciclosporin  in complete clinical
               response at 12 months
           Based on GRADE, the body of evidence was of moderate to low quality.
           8.3  Neuropsychiatry

           Treatment of NPSLE is determined by the underlying pathophysiology i.e.
           inflammatory or thrombotic. Corticosteroids and/or immunosuppressive
           agents  should  be  considered  in  the  former,  while  anticoagulant/
           antithrombotic treatment is favoured in the latter especially when aPLs
           are present. 50
           As mentioned earlier in  Subchapter 6.2,  two  systematic  reviews
           showed that CYC in combination with corticosteroids was more effective
           compared  with  corticosteroids  alone  in  NPSLE. The  treatments  also
           had a good safety profile. 68, level I; 70, level I

           Anti-epileptics  and  anti-psychotics  may  be  considered  as  adjunct
           therapy when indicated.

           8.4  Haematology

           Haematological manifestations frequently requiring immunosuppressants
           in  SLE  include  thrombocytopenia  and  AIHA.  First-line  treatment  of
           significant  lupus  thrombocytopenia  (platelet  count  below  30,000/
           mm ) and AIHA consist of moderate/high doses of corticosteroids in
              3
           combination with immunosuppressants (AZA, MMF or ciclosporin). 50
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