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Fibromuscular Dysplasia (FMD) (Dr. Gornik and Pam Mace RN - FMDSA - 12/14/11)

Heather Gornik, MD Cardiologist and Vascular Medicine Specialist,
Robert and Suzanne Tomsich Department of Cardiovascular Medicine,
Sections of Clinical Cardiology and Vascular Medicine

Wednesday, December 14, 2011 - Noon

Description

Fibromuscular dysplasia (FMD) is an uncommon disorder characterized by abnormal cellular growth in the walls of medium and large arteries. FMD is often found in women between ages 30 and 60, but may also occur in children and the elderly in some cases. Often the disease is hard to diagnose since many FMD patients do not exhibit any symptoms or findings on a physical examination or exhibit symptoms that can be misdiagnosed as due to another health condition. Treatment for FMD varies and can be tailored to treat disease severity. While there isn't a cure for FMD, it can be treated effectively. Dr. Gornik, a vascular medicine specialist and leader of The Fibromuscular Dysplasia Clinic at Cleveland Clinic and Pamela Mace, RN, President of FMDSA, nurse, and an FMD patient answers your questions.

More Information

Cleveland_Clinic_Host: Welcome to our "Fibromuscular Dysplasia (FMD)" online health chat with Heather Gornik, MD from Cleveland Clinic and Pamela Mace, RN from FMDSA. They will be answering a variety of questions on the topic. We are very excited to have them here today! Thank you for joining us, let's begin with the questions.


FMD General Questions

LindaK: Is it common for patients with FMD to have tortuous arteries? Are tortuous arteries a pre-cursor of FMD or are they caused by FMD?

Dr__Gornik: We have definitely seen as have other centers arterial tortuosity or "bendiness" and redundancy (extra longation) of arteries in our FMD patients. Dr. Jeff Olin at Mt Sinai in particular is very interested in this and I know he conducting some research related to this finding.

kptocs: Is the diagnosis of arterial tortuosity syndrome related to the diagnosis of FMD?

Dr__Gornik: Please see above answer. Many FMD patients have tortuosity of the affected arteries, and especially the carotid arteries. But arterial tortuosity, or “bendiness” is also associated with other blood vessel disorders, including Loeys-Dietz syndrome and a unique genetic syndrome that has been identified called the arterial tortuosity syndrome.

winonalisa: Should patients with FMD wear a medical bracelet?

Pam_Mace_RN: As a nurse I always recommend any patient with a disease wear a medical alert, but especially patients with FMD because the disease is considered so rare among so many physicians.

This would help to expedite your care in the event you were ever unconscious. There is a great website called Lauren's Hope that makes interchangeable bracelets and watches for the medical alert tag. www.laurenshope.com/

amintzer: Are there any other diseases associated with FMD?

Dr__Gornik: In the medical literature, there are some conditions that have been associated with FMD, such as certain blood vessel genetic disorders/connective tissue disorders. In some of these reports, it is not clear if the patient truly has isolated FMD, or if the artery lesions (narrowings, beadings) in the vessels that we have traditionally called FMD are actually due to some other vessel condition. For example, pediatric FMD which is generally the intimal type of FMD and causes tubular, smooth narrowings can be difficult to distinguish from certain forms of vasculitis or genetic conditions which can also lead to similar appearing arterial narrowings. So, as you can see this is a very complex issue.

kptocs What is the reason they call a psuedo anneurysm that? It sounds like it is fake or maybe not as bad as a real anneurysm. what is the difference and do you monitor or treat them differently?

Dr__Gornik: In the FMD world, pseudoaneurysms usually are the bulges or outpuchings that develop at an artery that has suffered an arterial dissection (or tear) or a trauma (such as a groin pseudoaneurysm that can develop after a traumatic puncture to a groin artery for an angiogram). It is a “false” aneurysm that does not involve all of the layers of the blood vessel wall, such as the aneurysms that can develop in the renal arteries or the brain in the absence of dissection. I know this wording can sometimes be quite confusing!

winonalisa: What are examples of "hypermobility"? Thank you...this is so helpful!

Dr__Gornik: Hypermobility refers to abnormal motion and ability to extend and flex the joints beyond the “norm”. Some patients with FMD may have evidence of hypermobility, but many do not. The same is true for patients without FMD, and in my opinion, I am not convinced that hypermobility is a “sign” of FMD for all patients.

Pam_Mace_RN: Dear Winonalisa, here is the link to a hypermobility scale called the Beighton scale, I think you will find it informative and helpful. www.hypermobility.org/beighton.php

mausimo32: Can a carotid artery dissection occur from a bad cough? They tell me that mine was caused from a bad cough I had a week prior.

Dr__Gornik: In most cases, we don’t know exactly what caused the dissection and patients and doctors try to “look back” and see if there was any forceful motion of the head/neck that could have caused it. So while it’s possible that “violent” coughing could have led to an artery dissection, I don’t think you or your doctors will ever know for sure. Sometimes the dissections unfortunatley can “just happen”.


Family Screening

Ronni: Ronni again, since mine (FMD) is genetic what are the chances my brother and sister may also have FMD? Thank you.

Pam_Mace_RN: Hi Ronnie, while I can’t answer your question I can tell you that I am only aware of less than about 10 families where multiple family members have FMD. We have a lot to learn about FMD and that is why research is so important. There is a paper that was published years ago called “The Genetics of Fibromuscular Dysplasia” by Dr Alan Rushton and Dr Kenneth Kidd – keep in mind we knew even less about the disease back then but you might find it interesting.

susankh: My children are (male) 21 and (female) 19 - I have been told this is genetic so are yearly physicals enough for them? with their doctor abviously knowing their family medical history and so listenting closely for Bruits and watching BP? should they also watch their BP closely at such young ages knowing theres a chance they have FMD too?

Dr__Gornik: Some good news susankh, among FMD patients, fewer than 10% of family members have FMD, so chances are very good that your children will not have that disorder. We don’t think FMD has a simple inheritance pattern, and again, this is an area in need of research.

kathleen: Are there any signs of FMD that children and grandchildren of FMD patients should be aware of?

Dr__Gornik: Great question kathleen. I tell my patients to let their family members (siblings, children) know about their FMD diagnosis and tell their family members to share this family history with their own doctors. Symptoms and signs to look for would be high blood pressure (especially if diagnosed at a young age), severe headaches, pulsatile tinnitus (“wooshing” in the ear) and of course bruits on physical examination.


Diagnosis, Testing of FMD

kaulrich: Can you please explain IVUS and how it is being used in patients with FMD?

Dr__Gornik: With the caveat that I am a non-invasive vascular specialist and do not directly perform the IVUS studies, I will answer your question. At our center, we use IVUS, or intravascular ultrasound, the most during our renal angiograms. IVUS allows us to take ultrasound pictures from inside of a vessel and help figure out if there really is a very tight lesion that is narrowing the diameter of the renal artery.

My interventional and surgical colleagues have found this helpful to determine if a renal angioplasty should be done. IVUS can also sometimes be helpful if a lesion on an angiogram looks atypical to figure out if there has been a tear in the artery, if there is thickening of the wall, or if there is a focal web. IVUS can also be used in other arteries (mesenteric). IVUS is not commonly used in the cerebrovascular arteries, but rarely our neurointerventionalists have used this. IVUS Is also used commonly in the coronary arteries, generally for atherosclerotic disease, and I have seen it used for the rare FMD patient who has had a coronary problem, such as a coronary dissection.

kptocs: I want to know how often screening for intracranial FMD/annuerisms should be performed. Is MRA a sufficient tool for screening for intracranial FMD? (in the setting of having FMD in both renals and bot internal carotids

Dr__Gornik: Great question. I have pondered the same on myself. I think we need to do more research to figure out optimal surveillance imaging for FMD patients. All of my patients with carotid or vertebral FMD will get an MRA usually to look at the intracranial vessels at least once. I have not been routinely repeating these studies if there are no brain aneurysms seen initially unless my patient has new symptoms such as worsening headaches, etc.

llbb: If a patient has newly diagnosed FMD in the renal artery, do you check all the other typical beds - carotid, vertebral, coronary just to be safe? What kind of imaging for those added screens? I know people do not want more radiation or contrast exposure than necessary.

Dr__Gornik: Another important question. For my practice, for a patient diagnosed with renal FMD, I will check the cerebral arteries (usually starting with a carotid ultrasound, but sometimes with a CTA or MRA depending on the specific situaation). If I see FMD present in the neck vessels, I always check for brain aneurysms/intracranial involvement (with MRA or CTA). I don’t routinely image the leg (external iliac) or arm (brachial) arteries in FMD patients, but I would do so if the patient had symptoms of claudicaiton (pain in the limbs with using them), pulse abnormalities, or bruits over these areas. I should emphasize that we don’t have a “national standard” or event consensus practice guidelines for exactly how to appraoch every FMD patient, so the above is just my approach.


Carotid Artery and Vertebral Artery FMD

mausimo32: I recently had a carotid artery dissection April of this year. I am stable but still have a 6.5 mm pseudoaneurysm/aneurysm that remains. Should I be concerned that I might have FMD? They say I don't because I don't have the string of pearl appearance. How often should one have a CTA to check the pseudoaneurysm/aneurysm? Thank you in advance for your time.

Dr__Gornik: Great questions. We see a number of patients such as yourself that have spontaneous vessel dissections. In my practice many of these patients end up having FMD either in the dissected artery or in another location such as the renal arteries. I think it would also be important to have you evaluated for other connective tissue disorders that can cause dissection. In many of the spontaneous dissection patients we never find a true cause for sure. As for follow up - we recommend periodic reassessing of the psuedoaneurysm, but in most cases they do not require any further intervention.

JK: My internal carotid arteries are both completely occluded. I have good collateral flow through my vertebrals. Do you see other patients with this degree of occlusion? I am only 37, so I am wondering what the prognosis is for someone like me. Is it possible to live a long time with this degree of occlusion

Dr__Gornik: Dear JK - for bilateral carotid occlusion in a 37 year old patient, is rare and we would be worried about things like bilateral carotid dissection or carotid thrombosis. I think in order to determine your long term prognosis it is important to find out the cause of why this happened.

You should see an experienced vascular specialist. As for the blood flow to your brain, by the very high level nature of your question, it sounds like the vertebrals are doing a good job providing your brain with blood flow. Patients can live from vertebral collateral blood flow.

cdsurvivor: Hello Dr. Gornik. Last year, I was diagnosed with a left CAD and left VAD with pseudo aneurism. A month after that, I was diagnosed with an abdominal aortic dissection. I am a young woman with young children. They mentioned to me possible FMD, but never followed up on it. I am only being montiored. My dissections are stable, but no healing has occured. If it is FMD, will I always have these, and are my chances or getting more, or dissecting worse higher?

Dr__Gornik: Thank you for your story. For others on the chat - VAD is verterbral artery dissection and CAD is either coronary or carotid artery dissection. It sounds like you have suffered 3 major artery dissections. This is possible with FMD but also seems somewhat atypical and I think it would be important for you to be evaluated for other vascular abnormalities, particularly some of the connective tissue disorders. If there is no physician in your area that specializes in FMD, you can also be seen by a medical geneticist that is specialized in diagnosing these disorders.

LindaK: Are you aware of any correlations between cervical and thoracic vertebral hemangiomas and FMD? Have you seen any cases of FMD occuring in the cervical or thoracic spine?

Dr__Gornik: Dear LindaK. No, I have really not seen cervical or vertebral hemangiomas in FMD, and FMD does not involve the spine.

jorler: I have registered through Harvard Brain for research to be done - hopefully many years from now--.. I have also put on the registry I had been diagnosed with FMD and the issues with all 4 of my vessels into the brain. Not only with the brain, yet is this an area where FMD specialist have been able to look into..

Pam_Mace_RN: Dear Jorler, Thank you for sharing this information as we have gotten questions from other patients about donating their body for research.

peppermintpatti55: I was diagnosed with carotid FMD, bilateral 50% a month ago after 2nd TIA in 3 yrs. My cardio and neuro are at odds in treatment. Cardio = plavix/baby aspirin; neuro = adds coumadin. Neuro does not agree with radiology finding but believes these are atherosclerotic ridges where clots are lodging. Isn't the radiologist trained more to read the CTA? He did note both FMD and atherosclerotic issues. Last week I had 2nd TIA and PFO closure. I am leery to take coumadin until FMD confirmed at Ochsner end of month.

Dr__Gornik: It’s hard to comment on your case without having a chance to review your records and examine you, but I think your situation really shows others how complicated some FMD cases can be, especially when there are multiple health issues “going on”, such as possible FMD versus atherosclerosis and a PFO.

JanaLynn: I have FMD of the carotid artery, is it common to experience dizziness/fainting when bending over and getting up from a seated position? Jan

Dr__Gornik: Dizziness is a common symptom we see in carotid FMD, but really fainting/loosing conciousness is extreme. You should discuss this with your vascular specialist in terms of how severe the FMD may be and also in terms of your blood pressure medications and whether your blood pressure is really dropping when you change position, making you more symptomatic.


Renal Artery FMD

Edna: Thanks for hosting this chat on FMD. My question is if one has bilateral FMD and has recently develop abnormal Kidney function results(BUN, Creatine, GFR) could the Renal FMD be part of the reason for the test becoming abnormal? Thank you very much.

Dr__Gornik: Renal FMD can cause renal failure, but in our experience, this is uncommon (though renal FMD causing high blood pressure or hypertension is very, very common). I think it would be important to also rule out other non-FMD causes of renal failure (rise in creatinine/BUN, or fall in GFR), such as diabetes mellitus, certain medications that can harm the kidneys, inflammatory kidney disorders. An experienced nephrologist should be able to help determine if FMD is the cause of declining renal function.

KathyC: 51 yr old white female with highly variable blood pressure (125 -200 systolic), recently diagnosed with renal fibromuscular dysplasia via CT scan. Referred to nephrology specialist for consultation regarding angioplasty. I am also concerned about possible FMD in other areas (particularly carotid). Is a more thorough full body exam recommended once FMD is discovered? Who would I go to? Cardiology, or ? Any recommendations for the Chicago area?

Dr__Gornik: Dear Kathy - For patients with renal FMD, at minimum, I do an imaging test (at our institution we usually start with a vascular ultrasound, but this can vary) to look for carotid FMD, as a significant percentage of our renal FMD patients also have carotid FMD. IF there is carotid FMD present, I would also recommend an imaging study of the brain arteries to rule out brain aneurysm (again at my institution generally an MRA test). I do a physical examination for pulses and bruits over the femoral or iliac arteries for evidence of lower extremity FMD and the brachial pulses for upper extremity FMD. If there are abnormalities of these vessels on physical examination, I would obtain an imaging study to confirm this. I don’t think a full body CT or MRI scan is necessary, but rather a targeted approach to the vessels that are generally involved with FMD.

As for the specialist or specialists to be seen for FMD, there are many specialties that take care of FMD patients, such as nephrologists, as you mention, but also neurologists, vascular surgeons, vascular medicine physicians, cardiologists, and interventional radiologists. Most important is to be seen by someone who is experienced in FMD. I’d always be happy to see you in Cleveland, and there are many other FMD centers in the US.

Pam_Mace_RN: As far as finding a physician in the Chicago area, If you go to the FMDSA web site and look under the support page you will see a patient volunteer listed from the area who might be able to help you with finding a local physician. I do not know of any doctors in the Chicago area but know that Dr Kolluri and Dr Alan Nadour from Prairie Cardiovascular in Springfield see fmd patients. To my knowledge a lot of patients that live in the Chicago area end up traveling to Cleveland to be seen at the FMD Clinic. I know there was a recent post on the FMDSA Inspire page about doctors in Chicage, you might want to take a look at that if you have not already.

mjesus: I had surgery Dec. 7 revascularization of the renal arteries. I had to put a stent in the right artery. What kind should I care?What kind of care I have with my body, my diet, exercise, etc.

Dr__Gornik: In general after renal revascularization you will need to be put on a follow up surveillance program which in most cases would be periodic renal ultrasounds for re-narrowing of the stent.

As for diet and exercise, there is no scientific evidence that shows any specific diet or exercise program can help FMD, though we think eating a heart healthy diet and getting regular aerobic exercise is important for overall cardiovascular health.

llbb: My local internist feels he has ruled out FMD based on the MRA appearance of my renal stenosis, which is proximal in my main renal arteries, and unifocal. Reading up, I see there are unifocal FMD renal cases. My stenosis in one artery is ~60 percent and quick math say only 15% of the cross section of the artery is open. I think this is significant.

Dr__Gornik: Yes - unifocal or intimal FMD the less common form, can present with a single lesion in the renal artery, but this is usually at the origin of the vessel where atherosclerosis develops. In most cases, an MRI should to be able to distinguish between atherosclerosis and FMD but sometimes this is challenging.

Your doctors comments reflect the fact that there is uncertainty if angioplasty and stenting benefits patients with atherosclerotic renal artery blockages. So I would say this is by no means straight forward.

Boppy: What is the best diagnostic technique for determining the extent of fmd in the carotid and kidney arteries?

Dr__Gornik: Unfortunately there is no uniform best answer. Invasive angiography remains the gold standard, but carries with it some risk. There are many non-invasive options to diagnose FMD (vascular ultrasound, CTA, MRA), but the best choice depends on the local imaging expertise in your area. At our center, for example, we generally use ultrasound as the first line test for renal FMD.

terriself: How do you insure that renal arteries are clear after angioplasty?

Dr__Gornik: At the time of the angioplasty, the interventionalist performing the procedure will look at the angiogram to see that flow is improved and narrowings looks angiographically better. At our institution, our interventional doctors and vascular surgeons also routinely use intravascular ultrasound (IVUS) or blood pressure measurements across the narrowing before and after the procedure to make sure they had a good result. Ultimately, we would like to see an improvement in blood pressure during the weeks to months after the procedure, but this can depend on facotrs other than the success of the angioplasty, such as family history, whether the patient has other risk factors for HTN (such as diabetes or overweight), and how long the patient has had high blood pressure. Usually after the angioplasty, a patient will be put on a surveillance program to recheck the renal artery periodically --- at our institution, this is generally done with are renal duplex ultrasound, but other centers may use other imaging methods.


Blood Pressure Control

artist: In March of 2010 after being diagnosed with FMD I had a renal angioplasty which immediately stopped the erratic HBP numbers that I had been experiencing. Sadly, FMD returned weaving its spell and the numbers have returned. I get on my elliptical which mostly works in bringing the numbers into a more normal range, and if need be I use Clonidine when that fails. Have you any other ideas of how to keep the numbers in a safer range as the HBP has caused some damage in the smallesst vessels in my brain. Cant afford to lose those or any other cells. Gratefully, Maxine

Dr__Gornik: Dear Maxine, first of all I would be worried that the angioplasty site has re-narrowed. Make sure you have follow up with the physician who had performed your angioplasty.

FMD_quest: Hello Dr. Gornick I have noticed that several days before my menstrual cycle my blood pressure will be increased I will get headaches him I'll be very agitated and I've talked to other FND patients who have had similar experiences are you noticing a trend here thank you

Dr__Gornik: Some patients have shared the same with me, and I think this is largely due to changes in fluid retention/volume that occur around the time of menstruation, as well as hormonal shifts. Even among patients without FMD, such as those who suffer from migraine headaches who do not have FMD, changes in symptoms according to the phase of the menstrual cycle is common.

Boppy: For someone with fmd, what is ideal blood pressure when sitting? Standing for 2 minutes?

Dr__Gornik: Optimal seated blood pressure is < 120/80 mm Hg. BP that is between 120/80 mm Hg and 139/89 mm Hg is called “pre hypertension.” Blood pressure greater than or equal to 140/90 mm Hg is high blood pressure/hypertension.

susankh: Dr and/or Pam, I have been told that I will need to continue BP meds for life and some form (probably 325 mg asipirin) therapy for the bood in order to maintain good vessel health. I am currently 7 month post bilateral carotid artery dissections with one side seeming to be healed and some healing in the other - my concern is for my physical self in teh future, will I be able to be as active as I was in the past? I was in the best shape of my life when this unexpectedly hit me....

Pam_Mace_RN: Dear Susankh, the first couple of years after my dissections were the hardest for me and I think it was due to the fact that I had 3 dissections that it took that long. That said I am doing well and stay active. It might be helpful to read some of the stories on the fmdsa web site. www.fmdsa.org/patient_support/stories

scottie: Do dissections heal over time and do aneurysms which are non-operable strengthen over time if the bp is controlled? thanks for your time and this opportunity to ask questions.

Dr__Gornik: Dear Scottie, yes dissections can often heal over time, but no, I’m afraid true aneurysms (such as renal artery, intracranial, or aortic), almost never regress or “shrink” in size. By controlling high blood pressure, the hope is that one can prevent the aneurysms from growing at a faster rate.


Coronary FMD

ErinB: Can FMD lead to heart attacks or death if undiagnosed? How is it diagnosed? Has the Cleveland Clinic begun using Premier Heart's Multifunction Cardiogram?

Dr__Gornik: FMD can rarely cause heart attack generally due to dissection or tears of the coronary arteries. That being said we have seen this only a handful of the nearly 250 patients with FMD we follow at Cleveland Clinic. There may be some more information coming out in the FMD patient registry data. We do not routinely screen the coronary arteries with imaging studies as it would be low yield and carries risk of radiation and false positive tests, but we always ask patients about coronary symptoms when they see us.

Pam_Mace_RN: Dr. Chris Buller at the University of Toronto is researching coronary FMD and last year spoke about over 30 coronary FMD cases that he has seen. I myself am aware of a few patients who have said they have been diagnosed with coronary FMD, but I agree that it is rare.

Ronni: Hi Dr. Gornik and Pam, Since I had a heart attack of the LAD back in 2006, my cardiologist had to find out more about FMD and then did another angiogram to make sure the stents were doing great and he assured me it is not FMD related. I just want to know how rare is coronary FMD. Thank you.

Pam_Mace_RN: Ronni - thanks for sharing your history and comments. Again coronary FMD is not something that a lot of patients contacting me state that is something they have. For patients on the chat that have been diagnosed with coronary FMD, Dr. Chris Buller I am sure would be very interested in speaking with you.


Connective Tissue Disorders and FMD

QueenBee: Hello Dr. Gornick I have read in several places that some patients believe that many of us with FMD have crossover diseases such as Ehlers-Danlos and Marfan's is there any scientific evidence to support those claims? thank you

Dr__Gornik: Not yet, just clinical observations that some FMD patients share clinical features with other disorders, such as the propensity for arterial dissections or aneurysms. I believe Dr. Nazli McDonnell at NIH has presented abstracts regarding a cohort of patients she follows with FMD This is an active area of research, including at my own center.

kaulrich: Dr. Gornik you have been seeing FMD patients for several years now, do you look at FMD differently now? Do you see more connective tissue overlap?

Dr__Gornik: Great question. One thing that I am seeing now that I have been taking care of FMD patients since 2005 is that for most of our patients, the disease process tends to be relatively stable, and although it can lead to a number of health problems (high blood pressure, dissections, very annoying symptoms such as the “swooshing”, etc.). I do think with medical therapy and in some cases angioplasty, this disease can be well controlled and FMD patients can lead full lives. So that is something I find really rewarding.

As for your connective tissue question, I do think FMD or at least the “beading” and “narrowings” that we call FMD is, for some patients, a manifestation of a more global artery/connective tissue problem. Some of our FMD patients have issues with hypermobility (while others do not), some patients have had artery dissections or aneurysms and may have aneurysms in vessels that don’t have any of the typical FMD changes (such as in the aorta). I think there is so much we have to learn in this arena, and I really hope the next 10 years brings more information.

One other thing I”ve already learned over these few years, is that while all patients with FMD have some things in common, every patient with FMD is unique and each patient may have other medical conditions that are not directly due to FMD but can potentially influence their symptoms. It is sometimes tricky figuring out what is FMD related and what is not, as all of are patients are at risk for other health problems (beyond FMD). Sometimes I see FMD become the “scapegoat” for all of a patient’s symptoms, and we have diagnosed a lot of other non vascular health problems in our FMD patients. Taking care of FMD patients and trying to learn more about FMD is definitely one of the most challenging and rewarding aspects of my work.

LindaK: Thank you for doing these chats. They are so helpful. Connective tissue diseases have been mentioned several times. Could you tell us the most common connective tissue diseases being researched and where would an FMD person go to be tested for these connective tissue diseases?

Dr__Gornik: I think it is important to say again that most patients with FMD that I see do not have many features of an additional connective tissue disorder. We do test our FMD patients with arterial aneurysms or dissections for the most common known connective tissue disorders - ctds - associated with this. This would be Ehlers Danlos Syndrome; Loeys Dietz Syndrome; Marfan Syndrome in some cases and new mutations acta-2 and smad-3.

In my experience however, this has not been very high yield. Thus, there may be a connective tissue or genetic disorder associated with FMD but I don't think current scientific technology can "test" for it. That is why we and others are collecting (Mayo Clinic) the bio-repository of FMD patients and family members.

terriself: Any connection between marfans, Klinefelters and FMD?

Dr__Gornik: See above, there are case reports of patients with Marfan sydnrome have FMD changes in their arteries, but I don’t know of a link beetween Klinefelter’s and FMD.

winonalisa: Can you say moretalk more about connective tissue disorders

Dr__Gornik: Dear winonalisa, please see above. This is obviously a topic of great interest among those on the chat, and also those of us who see FMD patients and notice some common features and overlap in some cases of FMD and other blood vessel related connective tissue disorders. This is an area of focus for future research.

LindaK: Can you tell us about the relationship between FMD and segmental arterial mediolysis (SAM) and how the two are differentiated?

Dr__Gornik: Segmental arterial mediolysis or SAM is a disorder that classically presents with an arterial dissection/tear in one of the abdominal vessels, especially the celiac or mesenteric arteries. It can often mimic FMD, and it can be hard to tell these disorders apart, though I don’t think of SAM as causing beading or narrowings of vessels, mainly dissections. SAM can affect men and women more equally than FMD that is primarily a disorder of women (in the adult form). Unfortunately, only a sample of artery tissue can make the SAM diagnosis (SAM refers to how the vessel wall media appears under the microscope), but we rarely get tissue specimens (i.e., only if a patient undergoes an open surgery or bypass), and we do not generally biopsy arteries in the abdomen for diagnostic purposes.


FMD Symptoms

kaulrich: What are the symptoms PAD in the upper extremities? How is PAD related to FMD? What test shoulD be done to check for PAD?

Dr__Gornik: Greetings Kaulrich. At our center we have seen a handful of patients with upper extremity FMD (generally involving the brachial arteries) and also some more patients with lower extremity FMD (generally involving the external iliac arteries in the upper leg/pelvic region). Most of the patients with arm or leg PAD have had no symptoms at all and were identified as having FMD on the basis of physical examination findings (such as bruits over the arteries on a careful physical exam) or because an imaging study was done that looked at the brachial arteries in the arms or the external iliac arteries (often seen on a CT scan of the abdominal vessels, for example).

In terms of arm FMD, we have a few patients who have actually presented with thromboembolic complications in the arm/hand.

FMD in the arms or legs could also cause claudication or exertional pain in the muscles of the limbs that comes on with use of the arms or legs (walking) and goes away quickly with rest. I have seen this rarely in the legs involving the external iliac arteries, but not the arms.

In terms of my approach to looking for peripheral artery/limb PAD, I don’t think it’s high yield to perform imaging studies (in my experience). I usually start with a really careful vascular physical examination, feeling the pulses and checking for bruits and if there abnormalities suspicious, I would go to an imaging test.

LindaK: What are the most common symptoms of FMD of the arms? What arteries are usually involved when FMD affects the arms?

Dr__Gornik: Please see above.

susankh: How is the throbing I am experiencing in my ear 7 months post dissection (as a result of FMD) related to the dissectio and will it go away? It comes and goes but it quite bothersome when it is happening

Dr__Gornik: Dear susankh, unfortunately throbbing pain and also swooshing in the ears is a really common symptom of FMD patients and especially those who have had a dissection. Be sure you have regular follow up for the dissection which would usually include periodic imaging to assess for artery narrowing or psuedo aneurysm. An audiologist might also be able to help you with some things to try to make things better. Pam, other thoughts?

Pam_Mace_RN: in my personal experience and from many patients I have spoken with who have had dissections, their symptoms seem to get better as the artery is healing. In my case it took a good year after the dissection and now only occasionally am I symptomatic.

myadvocate: Can you describe flank pain a bit. I have been having right flank pain for six months and seen many doctors with no diagnosis.

Dr__Gornik: Flank pain is pain along one of the sides of the back. It can rarely be a symptom of FMD, but it can also be due to many other things such as urinary tract infection, kidney stones, nerve problems and muscle problems.

Bri123: Hi Dr. Gornick, My left vertebral dissected in May causing a stroke and FMD was subsequenty discovered in my inner coratids, vertebrals, renal, iliac, mid brachial & radial. I have a lot of head/neck pain and would like to have a better understanding of what the pain is from. Does is mean the stenosis is getting worse?

Dr__Gornik: Dear Bri 123 - sorry to hear about this. You have what sounds like multi-vessel FMD. Post dissection headaches and neck pain are very common. In some cases there may be a narrowing of the artery or pseudo aneurysm but in other cases the headaches persist even after the artery has healed. I would suggest you be evaluated by a neurologist or neurosurgeon with focus on vascular neurology. A headache specialist may also have some good ideas as to how to help you.

myadvocate: Is the Swooshing a high pitched whine that beat with heart beats? Which I have but not diagnosed with FMD

Dr__Gornik: Yep. Sounds like you have pulsatile tinnitus. It is common among FMD patients (and very annoying I hear). But - it can also be due to a lot of other causes. Start by seeing an ENT physician and maybe having a carotid ultrasound.

Pam_Mace_RN: There is a great web site - www.whooshers.com - for pulsatile tinnitus. The website is very informative. Keep in mind this is not FMD specific.

terriself: Is there fatigue associated with FMD? I also have Sjogrens Syndrome?

Pam_Mace_RN: I do get contacted by a lot of patients stating that they are constantly tired. I can tell you since my diagnosis that I do require more sleep than I used to.

Dr__Gornik: I firmly believe that FMD in of itself should not cause total body fatigue. Some patients with FMD and carotid dissection who suffer from headaches or who have had strokes may have fatigue related to their neurological event. But, for patients with just beading in arteries we can't scapegoat the FMD as the cause of fatigue and other things such as anemia, depression, sleep apnea, thyroid disease and other conditions must be ruled out.

scottie: I have daily neck and posterior shoulder pain on the side of my int carotid and vertebral dissections. Could this be related? I have mild sublcavian FMD also. The pain is not related to exercise/activity. I have to take tramadol daily -having tried milder analgesics which have not worked.

Dr__Gornik: Neck pain is a common symptoms among patients with FMD who have suffered a dissection of one or more of the cervical (carotid or vertebral) vessels and could definitely be related. For patients with such significant pain after a dissection, follow-up with a neurologist, neurosurgeon, or neurointerventional radiologist is important to be sure there are no large pseudoaneurysms that have developed in the vessel wall. We have also worked closely with headache specialists to help find some treatments that can help out patients “cope” with their dissections.


Raynauds and FMD

winonalisa: Any indication that Reynauld's could be connected to FMD?

Dr__Gornik: Not directly but Raynauds is more common among women and FMD is definitely more common among women. So - some of my female FMD patients also have raynauds but I don't think in most cases there is any sign of upper extremity FMD.


Anticoagulation

Boppy: For someone having had at least 2 kidney artery infarctscaused by fmd, should warfarin be prescribed on a life long basis?

Dr__Gornik: Possibly but this would really need to be evaluated very carefully. In general, renal infarct is uncommon with FMD, but we have seen a few cases.

mjesus: I'm from Brazil, I have 53 years and just found out I have fibromuscular dysplasia, why do I have to take anti platelet(Clopidogrel) or aspirin for good?

Dr__Gornik: Welcome Brazil! I hope you are enjoying the sunshine! We definitely recommend anti platelet therapy for patients with carotid or vertebral FMD. This is to prevent stroke or mini stroke as the beads and narrowings of FMD can lead to micro clot formation. For isolated renal FMD, not every patient necessarily needs anti platelet therapy.

Boppy: What are the preferred meds for anti -hypertension and anti-platelet issues with fmd?Perhaps warfarin for a short time after the infarct?

Dr__Gornik: Dear Boppy. Choice of antiplatelet therapy (or need for anticoagulation) and specific blood pressure agents vary for each patient and clinical situation, so I can’t give a general rule.

mausimo32: After a Left ICAD and stroke I am on aspirin for life. 325mg....wouldn't 81 mg be enough?

Dr__Gornik: Good question. Some patients may not have complete suppression of platelet activity (aggregation) on low dose aspirin (81 mg), and for some patients, starting with a higher dose of aspirin is recommended or performing aggregation studies to see if the low dose of aspirin is adquate. Please discuss this with your doctor, especially if you are having side effects on the 325 mg dose (e.g., stomach upset).

peppermintpatti55: Is there ever any reason an FMD patient should be put on coumadin ... say if there is a dual diagnosis of atherosclerosis and FMD in the arteries?

Dr__Gornik: Most patients with FMD (generally we are talking about carotid FMD for antiplatelet therapy) can be managed with antiplatelet therapy. Coumadin is usually reserved for patients who have had a recent dissection, had a clot in one of the arteries or veins, or also have a clotting disorder or atrial fibrillation (problems not related to FMD). Again, each situation is different and care must be customized to each patient’s history. Similarly, most patients with atherosclerosis are managed with just antiplatelet therapy unless there is a compelling reason for coumadin.


Vitamins and Supplements

peppermintpatti55: Do you recomemnd any supplementals to take that can help strengthen artery walls, such as possibly an arginine complexes, etc.?

Dr__Gornik: There is zero evidence of benefit of any nutritional or vitamin supplements for FMD patients. A few years ago l arginine was studied as a treatment for atherosclerosis in the legs and it actually made patients worse. So - I would caution you in terms of the marketing of these supplements. A multivitamin certainly is not harmful.


Activity and Exercise

Tamara: Five days ago, I was diagnosed with FMD of the carotid and vertebral arteries. I don't have symptoms other than the infamous "woshing" sound in my ears. I am a very active 50 year-old woman. Will I still be able to do hot yoga, run, ballroom dance, and golf? What about getting massages or twisting my neck (side-to-side, back and forth) to relieve muscle tension? Thank you.

Dr__Gornik: Dear Tamara, I am impressed that you found this chat after being so newly diagnosed. In terms of cerebrovascular (carotid and vertebral) FMD that has manifest with the pulsatile tinnitus, I do hope you would be able to continue to be active. For most of our FMD patients, it is fine to continue aerobic exercise, dancing, golfing, yoga, etc. We generally do, however, recommend against intensive competitive exercise, heavy weight training, contact sports, and scuba diving. This is because carotid and vertebral FMD are associated with an increased risk of a dissection (tear) developing in the arteries. We also recommend against chiropractic manipulation of the neck and also, believe it or not, “herky jerky” amusement park rides or roller coasters.

That being said, for most FMD patients, the things that you describe enjoying should be OK. AS always, check with you doctor first (who knows the most about your medical history). Also, it is important to be sure you’ve had a test done to rule out the possibility of a brain aneurysm which can occur in some patients with carotid/vertebral FMD.

winonalisa: I was diagnosed with FMD of the carotid and vertebral arteries last week. What types of physical activities are patients with this avoiding? Massages or yoga? How about running or golfing?

Dr__Gornik: We generally recommend against activities that put heavy strain or torsion on the vessels. That would include:

  • weight training other than very light hand weights
  • contact sports 
  • diving 
  • sky diving 
  • chiropractic manipulation of the neck 
  • and believe it or not - roller coaster or other intense carnival thrill rides.

Pam_Mace_RN: I think each patient should speak to their physician regarding restrictions based on which arteries are affected; severity of disease; and if they are symptomatic.

susankh: i was an extreemly active person, exercising 4 days a week for approx 6-8 hours total, will I ever be able to resume some of that activity ? i did high impact aerobics, kickboxing, pilates, weigt lifting etc.

Dr__Gornik: Please see my previous answer but unfortunately I do think you will need to modify your exercise program somewhat.

Pam_Mace_RN: I can tell you I was very active and used to scuba dive, sky dive and run marathons. Dr. Gornik will no longer allow me to do these things although I did recently run a 3 mile race and have toned down my exercise regimen.

winonalisa: I do bikram yoga where the room is heated to 105-110 degrees. Would this be considered too intense and not advisable?

Dr__Gornik: I am honestly not sure and other factors than FMD, such as whether you have heart disease as well or are on many medications would also weigh in. Please touch base with your doctor about this.

Boppy: Are there any physical activities that are contraindicated for fmd?

Dr__Gornik: Please see above.


Children with FMD

amintzer: For children with FMD, what is the prognosis re later attacks?

Pam_Mace_RN: There is no long term data and I know of very few adults who were diagnosed as children. Children’s Hospital of Philadelphia (CHOP) has just gotten approval to enter patients into the patient registry on children. Hopefully, soon we will learn more and be able to share the findings.

Dr__Gornik: Dear amintzer, thanks for logging on. We also have entered some pediatric FMD cases into the registry. But, I think the addition of CHOP to the registry will greatly help us better understand the natural history of FMD. As you may know, the intimal type of FMD tends to be more common in children. Unlike the medial type, which we see most commonly in adults, the intimal type can have more aggressive narrowings. On a positive note, arterial dissection in my experience is less common in the intimal type.

Miggi: My 13 yo son was diagnosed with right renal artery stenosis resulting from Fmd Angioplasty was successful though he is still on bp med(atenolol and lisinopril) The right kidney is small and both the left and right kidneys are scarred.. His nephrologist believes the scarring of the kidneys is a result of past urinary reflux though tests incdicate reflux is not a problem presently.. Over the last 2 weeks he has been having left side back pain that comes and goes. Up to 7 on a 1-10 scale of pain. He has been sent home from school 4 times over the last 2 weeks because of this pain..I talked to his nephrologist the 1st time it happened, he did not think kidney related. He saw pcp after the 3rd time. No significant findings. Could Fmd of a different artery cause this pain? The FMD diagnosis just happened in late October so I am still trying to learn.

Dr__Gornik: FMD is an unusual cause of back pain. In the abdomen, FMD mainly affects the kidney arteries, as you know and the intestinal arteries. His renal arteries should be re-evaluated but if those are ok it is hard to come up with another obvious vascular reason for his pain. A urologist may be helpful or perhaps an orthopedic specialist if the pain continues.

Pam_Mace_RN: Dear Miggi - If you would find it helpful, I would be able to put you in contact with parents of children with FMD who may be a good resource for you.
Please feel free to contact me at FMDSA - 216.834.2410.


FMD Treatment

Boppy: Is there any means for repairing arteries affected by fmd eg by softening the arteries?

Dr__Gornik: We don’t have any known treatments that directly reduce the fibrous tissue or tissue overgrowth that leads to FMD. Blood pressure control, not smoking, keeping cholesterol in a good range, and preventing or treating diabetes, are all things that are important to prevent additional vascular damage and maintain vascular health in general, but these maneuvers have never been studied specifically in FMD.


FMD Research

EliC: What's up with research on FMD?

Dr__Gornik: Dear EliC – I can’t speak to all of the FMD research going on, but I can update you on the things in which I am involved. We (Cleveland Clinic) are an active participant in the US FMD patient registry, and the first two abstracts from the registry data were presented at the American College of Cardiology meeting last spring by Dr. Olin and myself on behalf of the registry team. I’ll let Pam give you more updates on that.

Pam_Mace_RN: This is a very exciting time for FMD research – the FMD patient registry, the bio repositories, the research in Rotterdam, the research in Paris and the NIH study are all separate research projects. FMDSA is funding the FMD patient registry and the research in Rotterdam (described below), but we do our very best to stimulate and cooperate with others who are doing research. Here is what I know: I am excited to announce that Children’s Hospital of Philadelphia (CHOP) recently received approval to participate in the patient registry and I hope to soon announce yet another center.

Dr__Gornik: At Cleveland Clinic, in addition to the FMD patient registry, we are collecting blood samples from FMD patients and family members and will ultimately be working with other investigators (including colleagues at Mayo Clinic) to help understand if there are genetic underpinnings to the disorder. We are also looking at the effect of FMD on Quality of Life among the patients we see here at Cleveland Clinic, and I am working with one of my medical students looking at the yield of clinical genetic testing for certain connective tissue disorders among FMD patients. Pam, any more updates on the registry or other FMDSA sponsored research?

Pam_Mace_RN: The update I received from MCORRP (Michigan Cardiovascular Outcomes Research and Reporting Program) the coordinating center for the patient registry just yesterday is that we now have over 500 participants in the registry! From our meeting with the Paris group lead by Dr Plouin, we were thrilled to find the Paris group has an FMD patient registry, which is different in many respects than our registry, and that the group is very interested in collaborating. For those of you who have been following our work, I have to tell you it was truly moving to have so many people from around the world interested in advancing FMD research come together. There is no doubt we will all benefit from the good work of this group and the growing focus on FMD research.

As mentioned above, in addition to the FMD Patient Registry, FMDSA is funding research in Rotterdam. The research is being conducted by Dr Marja Wessels (MD, PhD), clinical geneticist, Dr Rob Willemsen (PhD) and Prof J Kros (MD, PhD), pathologist at the Erasmus Medical Center. They are continuing their work on histopathology studies including immunostaining for TGFB pathway components to describe in more detail characteristics of FMD pathology. They hope to have the study completed any time now and we hope to have Dr Wessels speak and share the data at the FMDSA annual meeting in May, stay tuned.

The NIH study is lead by Dr. Naz McDonnell (who is on the FMDSA medical advisory board). My understanding is that the NIH is still excepting applications for participation and I have had patients tell me that there is currently a long waiting list. I don’t have an update but I am sure when Dr. McDonnell has findings available to share she will be happy to update everyone.

It is truly amazing how far we have come since FMDSA was incorporated in 2003. I would like to take the opportunity to thank all of our members for your support as none of this would have been possible without you.


More Information, Support and Annual Meeting

Ronni: Hi Dr. Gornik and Pam, Thank you both for doing this again. It means so much to all of us. I am getting really excited about the next upcoming Conference in May, 2012. Any information about the speakers that will be there? How about Dr. Nazli McDonnell? The Conference is always informative and fun. Thanks so much for all you do and hope to see you in May.

Pam_Mace_RN: HI Ronni. We already listed some speakers on the FMDSA website - www.fmdsa.org. And - we will be announcing additional speakers soon. We are hoping to have Dr. Wessels speak to the research being done in Rotterdam. As far as Dr. McDonnell she always has an open invitation to speak. We are excited to have her back as soon as she has more data to share.

winonalisa: RE: Mayo--Can someone recommend a physician there? I am a new patient and want to make sure I get connected to the best at Mayo. Thank you.

Pam_Mace_RN: At the Mayo Clinic - Rob McBane; Thomas Rooke; and also Dr. Sanjay Misra see FMD patients. The Mayo Clinic is also involved in the patient registry. Also Dr. Iftikhar Kullo at the Mayo Clinic is heading their bio-repository research.

jorler: Short version-- am I do everything to avoid the next stroke or at least to minimize the damage.. After a few TIA,S in 2001 II had a major stroke due to dissection of left carotid. After 2nd stroke rushed into surgery and stents added as at this time it was the only option. Ended up needing approx 6-7 inches as it continued to tear, then brain hemmoragh, then brain surgery and skull put into the freezer. With the next stroke in 2010 being the right carotid I was then diagnosed with FMD. Appreciate the information I am now getting with th esupport group and always open for an FMD specialist thoughts-- thx

Dr__Gornik: Dear jorler, your case is obviously a very severe one. I have seen some patients who had dissections and in whom the association with FMD was not immediately recognized. We are now making some headway thanks to FMDSA and others in terms of raising awareness of FMD as a cause of carotid dissection.

Pam_Mace_RN: We have gotten a lot of positive feedback about our new INSPIRE community. Here is the link for those of you who have not yet found us: www.inspire.com/inspire/group/fibromuscular-dysplasia-society-of-america%20/

Patients have the opportunity to start their own discussion topics and other patients have been jumping right in helping to share and answer their questions. You can find the link to INSPIRE from the home page of the www.fmdsa.org website.

Also - there are groups being started in different states and patients have had the opportunity to join these groups and meet with other local patients. You can also find that information on the website.

I know a lot of patients have shared that they find comfort and support at the FMDSA annual meeting. It is a room full of patients with the same disease who at the end of the conference have the opportunity to ask questions of the physicians and also share their experience with others diagnosed.

Here is the link for more information on the meeting: www.fmdsa.org/news/annual_conference

kaulrich: Hi winonalisa- Mayo is starting to recognize the need for a team of physicians for FMD. Many of us FMD patients see Dr. McBane- Vascular, Dr. Robert Brown, Neurology - Dr. Lynne Shuster- Women's Clinic

Dr__Gornik: I have a lot of respect for my colleagues “up north”; Mayo is a great FMD center, as is Mt. Sinai Medical Center (Dr. Olin).

mjesus: Thank much for your help

amintzer: Dr. Gornik - thanks so much for everything you do!!!

kaulrich: Thank you Dr. Gornik and Pam for taking the time to answer our questions. I wish you health and happiness in 2012.

Dr__Gornik: As always the hour flew by and actually we have gone overtime. Thanks for your terrific questions. Thanks to Pam Mace for joining us today and thanks to FMDSA for all it does for FMD advocacy; support; awareness and research.

Pam_Mace_RN: I would like to thank Cleveland Clinic for having me today and also all of the patients and our members who have made all of this possible. I wish you all happy holidays. Late breaking news - Miami Baptist in Florida just received approval and is now participating in the FMD registry!

Dr__Gornik: See you in 2012.


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Reviewed: 10/12

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