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| We had one extra for lunch. |
Recently, research papers have appeared from two different groups which both review the published cases to date and suggest treatment strategies. The papers originate from Dr Zalewski and Dr Tobin at the Mayo Clinic and from Dr Taieb's team at Montpellier, both of which have been heavily involved in CLIPPERS reporting and research. It is gratifying to see that there is substantial consensus on treatment which will certainly be helpful for the newly diagnosed.
The first treatment stage is intravenous methylprednisolone 1g / day over 5 days (Dr Taieb suggests up to 10 days if necessary). This should be followed by oral prednisolone 1mg/kg/day (Dr Taieb suggests for a month and Dr Zalewski suggests until expected clinical and radiological i.e. MRI, improvement is seen).
In the second phase Dr Zalewski introduces a "steroid-sparing" agent such as methotrexate or azathioprine or (one I haven't come across before, possibly as it is "15 times more expensive than azathioprine") mycophenolate mofetil. Dr Taieb suggests methotrexate in the first instance; I contacted him to ask why he doesn't use azathioprine (although he does recommend it if methotrexate can't be used for any reason). He pointed out that the reported CLIPPERS cases treated with azathioprine in the literature are far fewer and tend to be atypical.
The "steroid-sparing" agent is usually ramped up slowly to test tolerance and the oral steroid can then be reduced. Both authors agree that 20mg/day is the minimum steroid dose that should be maintained to prevent symptoms returning until the alternative drug has reached an effective dose level. Dr Taieb also suggests alternative drugs if methotrexate can't be tolerated: azathioprine, cyclophosphamide and hydroxychloroquine.
I have missed out a lot of detail in this summary (and I am not a doctor) but nevertheless these papers do, in my opinion, mark a step towards an accepted treatment strategy which is effective in the majority of cases. Of course this all assumes that an accurate diagnosis of CLIPPERS can be obtained in the first place. If the stage 1 treatment above fails to provide any improvement then the diagnosis is probably incorrect. In addition Dr Taieb suggests that if there is any relapse with oral prednisolone at doses above 20mg/day in conjunction with methotrexate then the case needs to be looked at very carefully again.
P.S. I should mention of course that taking any of these drugs is not without potential problems. So if anyone out there is facing choices over treatment I urge you to ask your doctors about possible side-effects both short-term and long-term so you can make an informed decision.
Read other articles in this series at Living With CLIPPERS.
Living With CLIPPERS by Bill Crum is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.
