Steroids - Short-term, medium-term or long-term?

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 :

1. maintaining the patient at that dose 
2. maintaing the patient at that dose together with another immuno-suppressant
3. 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.

CLIPPERS?

Regular readers will know that the individual CLIPPERS case studies coming out now seem to be about CLIPPERS in conjunction with other conditions. I think pure individual cases of CLIPPERS are not adding much to previous research, but studies with large numbers of subjects are much harder to organise, especially with the still relatively small number of cases.

So this new paper is about a case of Lymphoma following CLIPPERS. This  seems to be better argued (at least in my definitely inexpert opinion) than this previous one relating CLIPPERS to previous influenza. This new connection is slightly worrying, as the authors discuss possible mechanisms (that I don't pretend to understand) whereby this case of CLIPPERS was an early indicator of something worse. 

As ever, it is impossible to draw strong conclusions from a study of one patient and, given that this link has not been previously reported, it is certainly not common in the studies which have been seen to date. When I was being diagnosed, I was told some form of Lymphoma was a possible alternative diagnosis and crucially, that it would also respond to steroid treatment but only in the short term. In this paper, the steroid treatment seemed to become less effective quite quickly and MRI subsequently showed a very un-CLIPPERS like lesion, even though the early appearance was CLIPPERS-like. I held my breath for about the first 3 months I was on steroids but when my improvement was sustained, it became less likely to be Lymphoma. So is this case CLIPPERS becoming Lymphoma or early Lymphoma mimicking CLIPPERS? I tend to think it's the latter as diffuse Lymphoma is reportedly very rare and very hard to diagnose. But I am not a doctor so what do I know!?

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.