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Scleral strengthening in Marfan syndrome - Dr. Hagan?
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Scleral strengthening in Marfan syndrome - Dr. Hagan?

Connective tissue weakness appears to be at play in severe myopia in Marfan patients.  As you may be aware, there has been enormous excitement in the Marfan community lately based on research originating at Johns Hopkins using ARBs (and in some studies, ACEi's) like losartan to reduce TGF-b signalling and thus arresting or even reversing aortic dilatation.  

Since it's probable that the same underlying signalling issues are at play with Marfan-based myopia as with aortic dilatation, would you venture a guess on whether these drugs might also stop myopic progression and strengthen the sclera?  I understand that the eyes are very rich in fibrillin.  

I have some sort of connective tissue problem (not Marfan, but perhaps MASS syndrome), and I began using ARBs (valsartan) a few years ago, when this research first debuted.  I graduated to a maximal dose (320mg/valsartan/day) about a year and a half ago.  I don't know if this is wishful thinking or what, but my myopic progression and aortic dilatation both seem to have completely stopped (and aortic dilatation actually appears to be reversing to normal size).  But perhaps the myopia is just halted for the time being, or perhaps it's stopped just because I'm now 28 and it's not going to progress further.  

So, in summary, even if you don't know for sure, any guesses would be fantastic ... particularly whether you know if upregulated TGF-b signalling is responsible for scleral stretching in Marfan syndrome.

Thanks!

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I am sorry but I do not think I can render a valid opinion on this type of research. Perhaps a Marfan support group might have that information.

JCH MD
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