What do you view as reasonable alternatives to cyclophosphamide in the treatment of neuropsychiatric lupus?
Have you had experience with sirolimus in SLE?
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In general, I very seldom, if ever, rely on cyclophosphamide anymore to treat lupus in general, and much less neuropsychiatric lupus, with few exceptions. As above mentioned, we start off with corticosteroids, followed by a second-line agent, which can be azathioprine, CellCept, Rituxan, etc. We have newer drugs for lupus such as belimumab and other cytokine blockers; however, limited data as to their preventive or therapeutic effect in CNS lupus disease. True brain vasculitis in lupus is rather rare, and this cannot be diagnosed directly by just imaging although, it is mentioned routinely by neuroradiologists in their interpretation of brain MRIs and MRAs. CNS lupus is a heterogeneous disorder, which we still struggle to fully understand, ...
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“A reasonable alternative” to cyclophosphamide in NPSLE is an amazing statement that belies the reality of this state. We have to recognize that NPSLE includes underlying pathophysiology that includes inflammation of meninges, cerebral tissue, and/or vasculature that may or may not be separate from a thrombotic diathesis with or without aPL antibodies. The diversity of underlying etiologies limits our ability to conduct any sort of clinical trials of any value. NPSLE is classically excluded from large scale clinical trials conducted for new pharmaceuticals. The role of IVIg, rituximab, and mycophenolate is limited to few trials and a challenge to interpret in this context, but are worthy of consideration on a case-by-case basis. There is a ...
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There is peer-reviewed evidence that sirolimus blocks seizures in patients with tuberous sclerosis. We do have the experience that sirolimus helps all forms of SLE, including neuropsychiatric lupus. Our experience is consistent with the overwhelming evidence that mTOR activation is found in patients with lupus and tuberous sclerosis.
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CNS lupus is perhaps one of the most challenging diagnoses, especially for a community rheumatologist. Due to toxicity of cyclophosphamide, it is scary to start it unless I am convinced that it is a true vasculitis rather than multi factorial process including thrombosis/psychiatric manifestations. Thankful for mycophenolate to help reduce need for cyclophosphamide.
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