What is the role of cytotoxic agents in the treatment of CNS lupus?

Updated: May 04, 2021
  • Author: Pradeep C Bollu, MD; Chief Editor: Niranjan N Singh, MBBS, MD, DM, FAHS, FAANEM  more...
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Various steroid-sparing strategies have evolved for long-term use in systemic lupus erythematosus (SLE), including cyclophosphamide 0.5-2 mg/kg/d, azathioprine 1-2 mg/kg/d, and methotrexate 10-15 mg given once weekly with folate rescue, permitting gradual reduction or elimination of chronic steroid therapy. Higher dose ranges or dosing based on body surface area may be used for these medications based on the experience of individual clinicians.

All chronic cytotoxic regimens present substantial risks and should be followed only by physicians familiar with these agents. In acute, life-threatening illness, one option is to initiate cyclophosphamide PO or a single dose of 8-20 mg/kg IV, along with IV methylprednisolone. [44]

Jonsdottir et al reported that the majority of patients improved following rituximab plus cyclophosphamide. [45] The differential downregulation of anti-DNA of the immunoglobulin (Ig) G and IgA but not the IgM isotypes supports the hypothesis that cells producing pathogenic autoantibodies are preferentially targeted by the treatment. The fact that greater absolute numbers of CD19+ cells at baseline predict a less impressive clinical and serologic response suggests that more flexible dosing could be advantageous.

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