Fibromuscular Dysplasia

[firewatcher]

Just poking around the internet...again. Looking for anything that would explain my screwed up labs.

I found fibromuscular dysplasia: a condition that causes narrowing of certain arteries (stenosis,) usually renal (kidney) but sometimes carotid, causing low blood-flow to the brain. This would explain many of my lab results, and sounds like it could cause POTS. Have any of you been dxed with this?

I confirmed this morning that my Grandfather has renal artery stenosis and it looks like it could be caused by fibromuscular dysplasia, which is autosomal dominant in genetic inheritance--in English that gives me a 50/50 shot of having it.

Any experiences?

[juliegee]

Jennifer,

I'm very interested in the connection. My gyn is DXed with this and she suspects that I and other family members may have it. Another symptom is vascular blow-outs ( my term.) You may recall, I have one bro who needed his aorta replaced as it began perforating and another who suffered with a cartoid dissection. My gyn has had her cartoid dissect TWICE. She has no kidney issues and her BP is low. She is on a regimen of meds to control symptoms and feels very comfortable. Her docs are at NIH. She wants me to go there. She was unable to find help in ATL. She saw the same geneticist at Emory that I did.

Unlike other vascular genetic conditions, there is no definitive blood test for this one. Tell me what you've learned about this.

Julie

[firewatcher]

From what I've read, it is rare, but usually found in premenopausal women from 30-50 with untreatable high blood pressure. It is a genetic condition that is dominant. It is an abnormal cellular growth of the blood vessels and arteries. On the right scan, it makes the arteries show up like a string of pearls (the narrow parts between the pearls are the stenosis a positive scan is the only way to dx it.) It most commonly occurs in the renal arteries causing reduced blood flow to the kidneys and kidney disease, BUT it can occur anywhere and in the carotid, it will cut down or off the blood-flow to the brain. There is no cure, but there are treatments: blood-thinners and angioplasty. Sometimes, in between the stenosis (hardened, narrow parts) the artery can bulge due to hypertension and "blow-out." It presents in younger women as high diastolic pressure (mine was 121/101 this morning.) It can elevate the serum creatinine levels and lower the GFR. Certain meds need to be avoided (obviously.) I'm only very shallow into my research, but have a fax in to my doctor since my Dad confirmed that my Grandfather has it.

I don't know much yet, so cross reference everything!

Julie, I'll email you if I find out anything.

more info:

http://en.wikipedia.org/wiki/Fibromuscular_dysplasia

[juliegee]

Jennifer-

I think you may have stumbled upon something. If your grandfather is positively DXed.... you definately need to pursue this!

Just to clarify, not EVERYONE with fiibromuscular dysplagia has high BP & renal stenosis. My gynecologist does not. Her two cartoid disssections were her wake-up call. I forgot all of the meds she takes, (something to keep BP low even though hers already is) but the one I found most interesting is doxycycline. She takes it for ten days and then stops for two weeks and then takes it again for a 10 day period, etc. Doxycycline is also being used by EDS, Type IV, folks as a preventative for vascular blow-outs. I don't think anyone knows how it helps, but it does. Since m gyn's been on this routine; she's been fine.

In addition to my 2 brothers, I have a nephew (8 y/o) with renal stenosis. I googled autonomic dysfunction and fibromuscular dysplasia and I got tons of hits. Very interesting!

Please keep us informed about what you learn.

Hugs-

Julie