It is now fairly well established in the research literature that for many (most?) people diagnosed with CLIPPERS, high-dose intra-venous corticosteroids (usually Prednisolone) followed by lower-dose orally administered treatment improves the condition. What to do after this initial treatment phase and how to manage the condition in the medium and long term is more debatable. What seems clear, is that some kind of continuing treatment is required. The most common reported variants involve tapering the steroid dose to a low level and then either :
- maintaining the patient at that dose
- maintaing the patient at that dose together with another immuno-suppressant
- tapering the steroid down to nothing and maintaining on another immuno-suppressant
A new case study discusses these issues in the context of a 63 year old man diagnosed with CLIPPERS. This patient was treated with option 2 above (80mg/day Prednisolone) and then Methotrexate (2.5mg/weekly). However he had complications on Methotrexate and was moved to Azathioprine (100mg/day) and Prednisolone (now 20mg/day). On this treatment, the patient had a good response and no CLIPPERS relapses for 18 months (and onward).
The interesting thing about this paper, is the authors consider the treatment in the context of somewhat related disorders like cerebral vasculitis. There, option 2 is maintained for 2-5 years to prevent relapse, and they suggest a similar treatment strategy may be necessary in CLIPPERS. Now, this week is my 2-year anniversary of first CLIPPERS symptoms and 1-year anniversary of coming off Prednisolone entirely (option 3 above). The authors of this paper also say that successful steroid discontinuation in CLIPPERS has not been reported. My 1-year off-steroid period is too short to be called successful, as others have had relapses after longer periods without Prednisolone. So caution is required, but so far, so good.
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.