Saturday, 5 October 2013

CLIPPERS: State of the Nation

Greeek Cat on Patrol
The third, and most important, paper that appeared over the summer is to be published in the journal Clinical and Experimental Immunology. This paper, entitled CLIPPERS: Review of an increasingly recognized entity within the spectrum of inflammatory CNS disorders, is an up-to-date and substantial review of CLIPPERS in terms of definition, patients and treatments. I'll just pick a few things from the paper to talk about here. 

One is that the "pathogenetic concepts in CLIPPERS are principally still based on assumptions and speculations" which means that the cause and mechanism of CLIPPERS is still unknown.The authors emphasise that finding a unique cause is necessary for CLIPPERS to be confirmed to be a unique condition (as opposed to a strange variant of some other condition).

On the evidence so far, the authors say that the average age when CLIPPERS appears in is between 43 and 53 (this is only the average and obviously there are people much older and much younger who have CLIPPERS). They say there is some evidence that men are slightly more likely to be affected - or at least reported. These figures are based on more cases than were available when I speculated on who gets CLIPPERS in January 2012. Interestingly they also claim that the "clinical course ... seems to be relapsing-remitting". Although I progressed fairly slowly (over a few months) before treatment, I was definitely getting worse and didn't get as far as any remission before treatment. I'd be interested to hear whether anyone out there had remissions independent of treatment.

Also discussed are the various treatment strategies, focussing on the use of corticosteroids with or without additional immunosuppressants. Interestingly the authors note that the success of the steroid sparing agents (like Methotrexate, Azathioprine, Cyclophosphamide and Rituximab) when used in the absence of steroids has not been proven. It will take more longer-term patient monitoring and reporting to figure this out. I've now been on Azathioprine-only for over a year and OK so far ....

Finally, they note 56 cases reported in the literature. Presumably more cases than that are out there in the wild (including me!). It's hard to estimate the true number of cases - is it twice this number or ten times it or more?

This paper as a whole is a useful and detailed summary of where CLIPPERS is at in 2013. It also confirms there is still a way to go in terms of understanding, diagnosing and reliably treating the disease.

Read other articles in this series at Living With CLIPPERS.

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