Long-term CLIPPERS

You can't escape it!

Just a quick post this time. I've been reading this recent paper about outcomes a bit more closely (see the last post for an overview). One of the 12 patients really stands out - a boy age just 13 years old when symptoms appeared (patient 5). This is much younger than most other cases but the really surprising thing is the duration of illness. He is listed as having had CLIPPERS symptoms for 408 = nearly 34 years; so those of us who think we have something new will have to take a step back and realise CLIPPERS has probably been around in unrecognised form a lot longer. This patient was recruited via a Multiple Sclerosis centre so perhaps had been regarded as an atypical MS patient for years. Unfortunately he also had the most relapses (12) over the follow-up period but was not treated with cortico-steroids. Interestingly he went for 280 months = 23 years without any relapses according to the graph in Figure 2. So all this suggests that CLIPPERS is survivable in the long-term but that appropriate treatment is key to keeping it at bay as far as possible ...

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.

News Flash

The most important CLIPPERS paper of the year so far has just appeared. It's called "Long-Term Outcomes of CLIPPERS in a Consecutive Series of 12 Patients" by Dr Taieb and colleagues. I haven't had time to digest this paper properly as it is quite involved (and I'm not a doctor) but some very brief points from a first reading.

Back In Black

CLIPPERS Long-Term
(As usual, please note I'm not a doctor so don't take this as gospel.)

The paper focuses on the pattern of relapses in 12 people diagnosed with CLIPPERS, treated with cortico-steroids and followed over months or years. Dr Taieb says that "Secondary progression was not observed" - in Multiple Sclerosis secondary progression means "sustained build up of disability, independent of any relapses". So this study suggests that there isn't an underlying disease process continuing to cause damage after symptoms have been treated with cortico-steroids. This is good news.

Relapses
Patients who remained on steroid doses at 20mg/day or higher tended not to get relapses but patients who did get relapses continued to respond to repeated high doses of steroids. Dr Taieb found that early treatment of relapses was important to prevent worsening of symptoms during the relapse or continuing problems. So I guess it's good news that CLIPPERS remains treatable but not so good news that it can return after the first episode. We have to remember though that this is still only 12 patients so this paper represents an important step along the way rather than a definitive study.

Still Missing
One important missing piece of the puzzle is the effectiveness of alternative immunosuppressant therapy. Three of the patients described by Dr Taieb were moved on to Cyclophosphamide and one on to anti-CD20 (Rituximab) treatment with one subsequently relapsing. Of particular interest from my point of view is that there is no data on the long-term effectiveness of Azathioprine although there is some existing evidence (from other smaller studies) that Methotrexate can be effective.

I Am An Experiment

I have been on < 20mg Prednisolone / day since November 2011 and since June 2012 I have been taking only Azathioprine and am still well. So I guess we'll see what happens ....

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.

Another Quick Update

Things have been quiet recently which is why there's not been an update for a little while. Anyone who spent June in the UK will know it's not just because I've been out enjoying the summer sun!

I definitely couldn't do this with double vision ... (Prometheus, IMAX 3D)

I've been pleased to hear from another "CLIPPER" by email this week - that makes at least four since I started writing this blog which shows that CLIPPERS does exist in the real world outside the research papers and is being diagnosed.

I've also been Prednisolone-free for three weeks (but still on Azathioprine). I haven't noticed any change in CLIPPERS symptoms (I don't have any to speak of at the moment thankfully) but have been getting some stiffness in my legs - hips and knees mostly. This doesn't feel neurological in the sense that it isn't like the "locking-up" or balance problems I had last year. It's more like you might feel after you'd run up a steep hill and most noticeable after I've sat down for a long time - could just be middle age creep. I did briefly Google this association with Azathioprine and found some similar (non-CLIPPERS patient) reports. But on the other hand it's the way of the interweb that you can usually find a report of anything you want - if I suddenly developed a wart (or a potato!) that looked like David Cameron I'm sure somebody somewhere will have reported a similar association with Azathioprine. I also found reports of people getting joint/muscle problems when (or even after) coming off Prednisolone although I was a on very slow taper so I don't know if it was that either. Anyway add to the list of things to ask the doc next time I see him, but I obviously don't want to make the mistake of confusing correlation with causation.

I'd also like to thank the slowly growing readership (especially the ones who come back for more!) - hopefully some of the stuff here is still either interesting or useful.

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.