Takayasu’s Arteritis: Rare diseases Day 2021

text reads: living with takayasu's rare diseases day. two images of artie looking frustrated, on a green and brown collage background.

Hey pals,

I wasn’t diagnosed last Rare Diseases Day, so I really wanted to grasp the chance to talk about my Takayasu’s Arteritis with you on my blog. I’ve mentioned bits and pieces here and there like on Instagram or my Youtube Channel, but here I’m going to go into what Takayasu’s is and what that means for my daily life. I also have a selection of other Diagnoses which affect my treatment and flares, so I may talk about that a bit as well.

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I don’t want to go through my story again of how I was diagnosed as it was pretty intense, I did film a video explaining it all before I was officially diagnosed that you can watch, My Doctor Didn’t Believe Me. I will also be making a video specifically about the scans I had to diagnose Takayasu’s if you are interested, subscribe to my channel.

Now, onto Takayasu’s Arteritis. Pronounced Tah-Kah-Yah-Sooz Arh-Tuh-RIE-tis. Not Arthritis, Arteritis. A rare type of vasculitis that causes blood vessel inflammation in the large vessels (mainly arteries but occasionally medium sized ones as well) it most commonly damages the aorta and is often seen in the aortic arch and the main branches off it. The aorta is the large artery that carries blood from your heart to the rest of your body. It is also known as Pulseless Disease or Aortic Arch Syndrome as well as these are the main signs and symptoms of TAK. Easier to say as well, I guess for some people.

Takayasu’s can lead to narrowed or blocked arteries, of which I have two narrowed arteries that lead to each arm, but it can also lead to weakened artery walls that can bulge and tear which is called an aneurysm. Before I was diagnosed, I thought an aneurysm was something totally different, I thought it was like a blood clot/stroke. Takayasu’s can lead to pain in the limbs but most commonly arms, chest pain, high blood pressure, and even heart failure or stroke. Since joining some online communities for people with Takayasu’s, I’ve found that many people were not diagnosed until after a stroke or needing life saving heart surgery, if you watch my video linked above about my story you’ll see that in many ways, I got lucky and was diagnosed before something like this could happen, but not before permanent damage was already done.

Many, if not most, of us need medications to control the inflammation caused by our immune system attacking our body tissue, to prevent complications or further damage being caused.

There are two stages to Takayasu’s. Stage one can be impossible to identify, and as I was already dealing with other health issues for the years leading up to my Takayasu’s diagnosis, I don’t think I would have been able to find it earlier if I tried. Many people, but not all, will feel unwell with fatigue, muscle and joint aches and pains, mild fever which sometimes involves night sweats, and unintended weight loss. I have hypermobility syndrome and have dealt with muscle and joint aches and pains for years and years as well as fatigue. I never had a fever, I never lost any weight, but I did have night sweats on occasion. These are also symptoms of Crohn’s disease, which I was diagnosed with a year before Takayasu’s.

We were only able to eventually figure out my diagnoses because I had entered Stage 2. This is the stage that inflammation causes arteries to narrow, so less blood and oxygen and fewer nutrients reach organs and muscle tissue. Commonly signs are: weakness or pain in limbs with use, lightheadedness/dizziness/fainting, headaches or visual changes, memory problems of trouble thinking, chest pain or shortness of breath, high blood pressure, diarrhea or blood in stool, anaemia. I’ve had chronic headaches for years, the diarrhea and blood in my stool was crossed off by IBS/Crohn’s, I’ve also always had a dizziness about me often contributed to my hypermobility syndrome, my BP has never been that high and used to be relatively low… the only symptom I could go off was that my right arm hurt when I used it for anything, this is known as claudication. Washing myself, brushing my teeth, holding something, patting my dog… repetitive movement set my muscles on fire but it was often put down to hypermobility syndrome (as talked about in my video) or pinched nerves, but I had so many MRI’s, one of my entire spine, and not a single pinched nerve. I also had a nerve conduction test which came back perfect. Besides this, the only other thing I had noticed is that any time someone tried to take my blood pressure from the arm that hurt (my right arm) the machines never worked. I always had to tell them to use my left. Before my abscess draining surgery in November 2019, I’d just been having a panic attack over the cannula they were struggling to find a vein for, was sedated and was mildly aware of how much the cuff hurt my right arm and that I’d lost all feeling because it was squeezing so hard and it was lucky I managed to get out something like, “no you need to put it on the other arm, it won’t work,” before passing out. I woke up with bruises all over my right arm but they must have swapped over after I knocked out. This is what made my GP stop and think, and found I was almost entirely lacking a pulse. (This isn’t mentioned on all online resources, but it is on the Vasculitis Foundation’s page under symptoms and diagnosis.) Thanks to him, I made it to the right people and got diagnosed.

For some statistics, Takayasu’s (Also known and called TAK) is often diagnosed in AFAB people (9 out of 10 patients to be specific) but I have also come across some AMAB people too, between the ages of 15 and 40. It has affected people outside this age bracket too, but this seems to be the most common time frame. It is also more commonly diagnosed in Asian people and one study also specifically says in Latin American people. I have seen a few different estimations on how common this disease is but the Vasculitis Foundation and Rheumatology.org state 1 in 200,000 people. This leaflet says a UK study showed TAK affects 1 person per million per year, this is likely UK statistics. It also says 2 out of 10 people with TAK ‘get better’ after one episode and do not require long term treatment. It is currently understood to be an autoimmune disorder which occurs when the body’s natural defense system mistakenly attacks healthy body tissue. It is not thought to be genetically inherited but there is something thought to be within genetics and DNA that makes TAK more likely. TAK was first described by Dr Mikito Takayasu in 1908 (which I found out as I wrote this! More info on this under ‘History of Takayasu’s Arteritis’ here) For people who find videos more helpful for learning information, check out this Webinar by the Vasculitis Foundation on Takayasu’s.

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Diagnosing TAK is difficult as it shows differently in each patient. Vasculitis.org.uk has some really useful info about each scan with images so I recommend checking that out. Not everyone will have raised inflammation in their blood tests (ESR or CRP), or show active inflammation on a PETCT scan. The most accurate diagnosis is a biopsy but it is very risky to do on such large vessels as the aorta and is not usually done unless the patient is undergoing vascular surgery. In my regular physical exams, my pulse is manually checked and listened to all over my body. I’ll have you know, my left foot has a great pulse! But, next to none in my right arm. This is where the other name comes from, the “pulseless disease”, because the blood pressure and heart rate are different across limbs depending on how much damage the arteries leading to them have sustained. 90% of patients with experience one or more arteries narrowing and rendering their limb pulseless, or nearly. They also listen for sounds called ‘bruits’ which can be heard over large arteries with a stethoscope, as far as I know, I do not have bruits. You often need multiple kinds of imaging scans done for diagnosis and to find the extent of the damage done so far. An ultrasound can find narrowing in limbs and neck, MRI/As with contrast can find narrowing in the aorta within the body, PETCT is for active inflammation but doesn’t really tell you much else and needs radioactive sugar water to work which isn’t great for your body and health. There are a few other scans, but I’m not entirely sure what they are or how they work as I’ve not had them done but there are more details in the Vasculitis Foundation site under Diagnosis. Also the Wiki page on this can be helpful for people totally new to health care as it links many key terms to help with making understanding the basics of testing and body parts included easier.

When it comes to treatment, there are a few options, but the general goal is to decrease inflammation (often by suppressing the immune system) so the disease slows or stops progressing. Many people have to take steroids and find it difficult to fully come off them as their inflammation rises and their symptoms return. I am on month 9 of steroids and I am slowly tapering down. Steroids are not a fun drug to be on, I’m also planning to make a video talking about my experiences on them as this is the third time I’ve had to take them and the longest. Previous times, I’ve been on them for 1-3 months at a higher dose. Another medication family often used is Immunosuppressants. They suppress the immune system, like steroids, but have less side effects and are preferred over steroids if possible. This includes Methotrexate (often used for Arthritis, my Gran is on this as an injection) Azathioprine/Mercaptopurine (I’m on Mercaptopurine), and Mycophenolate Mofetil. I believe all of these are also known as Chemo drugs. The third family of drugs are Biologics, which I am also on and are also to suppress the immune system! Apparently, “those with severe disease who don’t respond to traditional treatments may be prescribed biologics”, but in my case, I’m relatively complex with multiple autoimmune disorders I think I had less choice. Biologics are complex proteins that target certain parts of the immune system to control inflammation. Commonly used are Tocilizumab (which I was almost put on, but apparently is very rarely use for Crohn’s patients due to complications) and Infliximab which is commonly used for Crohn’s patients as well. But I was put on Adalimumab (find out more here in this video about this drug and my personal experience) for Crohn’s before I was diagnosed (literally two months before I was diagnosed) so we decided to just keep it up and see how I went and in September we upped my dose to weekly instead of every two weeks, and then changed the brand from Imraldi to Amgevita as my Rheumatologist had been doing some reading of research for TAK and Adalimumab. In April, it will be my 1 year on Adalimumab and I’ll be making and posting an update video to my channel. Some people also need drugs to lower Blood Pressure/Blood Thinners to help reduce the risks of blood clots. The other option is surgery, which is reasonably risky. If you have surgery with active inflammation, it is likely to not work permanently and possibly occult (block) again, but many people have to have surgery because it is life or limb threatening. I’ve been told that if I start to permanently lose feeling in my right arm and it starts to look very weird (yellowy translucent because there’s no circulation) I need to contact the Vascular surgeons for emergency surgery to save my arm. It can also be to repair aneurysms. There are also Revascularisation surgeries where a blocked artery is bypassed, usually with a vein graft from somewhere else in the body, usually a leg. I follow someone on Instagram who had this surgery, Susana Ramírez from Spain. There are also Percutaneous Transluminal Angioplasty (PTA) that aim to open narrowed arteries by inserting an inflating balloon to stretch the narrowed section, which I think Susana has also had. The Vasculitis.org.uk site has a lot of really detailed information on that the other sites I have used lack so I highly recommend looking at this site if you want to do further, detailed reading. The Rare Diseases site has some information on clinical trials as well, for meds and TAK.

Due to the nature of these kinds of medications, there are things you have to be careful of and keep an eye on. Lowering the immune system can make you more prone to contracting colds and flus which is why I’ve had to be extra careful during the pandemic as I am more likely to catch it but also have complications that threaten my life. There is potential for bone loss (especially with steroids) which is why I’ve spoken to an NHS dietician and now take a high dose calcium vitamin as well as vitamin D (helps the body to absorb the calcium). You need regular check ups with your doctors to keep an eye on these things. You can also become anaemic, which I was briefly the year before I was diagnosed with TAK for 4 months I was on a prescribed high dose of iron tablets and continue to take store bought. We are also recommended to get the flu jab, pneumonia vaccination, and/or shingles vac, as this helps reduce the risks. We cannot take LIVE vaccines so if we want to travel somewhere that often needs a LIVE vaccine before you go, you have to have a chat with your doctors about this. I’ve not had the chance yet as I was diagnosed early in the Pandemic.

The main team for people with TAK are Rheumatologists (joints, muscles, immune system) they are in charge of your meds, a Cardiologist (heart doctor) I don’t yet have one of these, and a Vascular surgeon (veins and arteries) who I do have and have met a couple of times, this was the man who sent me for the ultrasound that found the narrowing and really started the diagnosis process. My vascular surgeon discharged me but said that I can contact his secretary if there are any urgent worries that may need surgery. I also have my Rheumatologist’s secretary’s email to send her direct emails about new symptoms incase they are linked to my TAK or my other conditions that are also managed by Rheumatologists (hypermobility syndrome and fibromyalgia). I also send her research studies that have been shared in the TAK Facebook groups I am in, as she seems to be taking an active role in researching and understanding this disease more. I found one that states Crohn’s and TAK are very similar in genetic (???) shape/appearance, sorry can’t remember the right terminology. There is a Crohn’s vaccine (called MAP vaccines) in the works that is nearly ready for the public and this could mean one for TAK could be developed if they really are as similar as it is currently thought. The vaccine isn’t a cure but it is thought to be a way of making more severe symptoms and damage, minimised and be less damaging, which is always good.

One of the less discussed symptoms is Carotidynia, pain and tenderness over the carotid arteries (in your neck) which is found in 25% of patients. I have experienced this symptom on physical exertion since my mid-teens. It becomes so painful it is hard to breathe, I have to stop what I’m doing and grip my neck. It is recommended to refrain from extreme cardio like HIIT or circuit training, as it can aggravate these symptoms too much. Exercise is recommended to continue as it can help widen narrowed arteries, or encourage the development of capillarys (small veins which can help make up for narrowed arteries with blood flow) so they recommend regular weight training and gentle cardio.

It is thought that the majority of TAK patients will continue to have relapses and remissions throughout life, but it also appears that for the majority of patients it ‘burns out’ after a period of 2-5 years. There isn’t a lot of information on this, sadly, as I would love to know more. When I was diagnosed in June 2020, I had already been experiencing active disease for two years, and this will be year three. I hope I follow this trend of burning out at 5 years but I’m also aware (because of other conditions I have) this may not be a reality and will be a lifelong condition I will have to keep an eye on and take meds for. Up to 25% of patients with TAK are unable to work due to their condition. Hello, that’s me. Or, I can’t work a traditional job due to my chronic illnesses. The mortality rate is low for TAK, with early intervention, careful monitoring and treatment. In the US and UK there is 98% survival rate of ten years. Most discussion on survival rate tends to be between 5-10 years from diagnosis, I assume this is because it is so rare and caught at such varyingly different stages it can be hard to say past 10 years. Mortality linked directly to TAK is often a heart attack, heart failure, aneurysm rupture, stroke or kidney failure. Not everyone with TAK will find steroids helpful for their condition, and due to having been on two high dose short term courses in 2019 before my TAK diagnosis, I could give a definitive answer to my Rheumatology team that steroids definitely helped the pain in my arm, which is a good thing.

Having Crohn’s and TAK at the same time is good and bad. Good, as there are a few meds that are used for both diseases. Bad, because there are meds for each disease that don’t work for the other, or worse, cause harm. One biologic used in TAK can cause bleeding in the colon for Crohn’s. So I was lucky to be put on the meds I was put on. Adalimumab is less often used in TAK but it has been used and not caused further harm. We are seeing how things go. Aspirin is also commonly used in TAK but having Crohn’s means I can’t take this drug either as it is a NSAID which can aggravate Crohn’s and cause inflammation and bleeding. My Gastro consultant said to me that once you have one autoimmune disorder, you are more likely to develop others, so even though TAK is rare it isn’t entirely a surprise. Also, my Gran has a couple types of vasculitis (Polymyaldia Rheumatic, she may also have a Large Vessel Vasculitis like TAK called Giant Cell) which means my genetics could have been predisposed to developing something similar and it was just the way the shuffle of DNA landed. Funny enough, the Wiki page for TAK actually has quite a detailed section on the genetics with linked references.

erythema nodosum
ID: Artie’s legs, three angles from the knee down, showing many round dark bruises. Erythema Nodosum July 2019.

As you may be able to tell, this information is spread about across different sources. None of them are the exact same, and many of them miss things from their lists. My first searched before I knew I had TAK was on the NHS site under Vasculitis. This one mentioned painful, numb or cold limbs which was the first time the idea of limb temperature came up, it’s not really mentioned anywhere else and I’ve always had bad circulation to my hands and feet, they are often always cold and become so painful when it is winter and I have to go outside (walking to Uni in Feb-March was the worst, it was like walking on glass) and may have a link to Raynaud’s Phenomenon. This page also recommends the Vasculitis UK site for further info, so that’s where I went next. It is important to use references with good and trustworthy information for the beginning of your journey in understanding TAK for yourself or for a friend/family member. A page I came across as I was researching for this post is by Patient.info (the site my GP surgery uses for appointments and prescriptions so I know it is trusted) and this is the first reference to a certain pain I experience with over exertion of my arm, a ‘tenderness overlying the affected arteries’. If I push myself too hard when weight training, I feel pain over my brachial artery, which is the narrowed artery in my right arm, right above the inner elbow bend. It also mentions back pain due to Aortic involvement which I have never read before, and is more specific about the types of rashes linked to TAK. Other sources have mentioned rashes but never a specific name or description of it. This site specifies erythema multiforme and induratum. I had Erythema Nodosum in July 2019 which was thought to be my Crohn’s (as 1 in 7 people with Crohn’s develop this) but I’ve also read it can happen to people with Medium Sized Vasculitis. Muscle wasting is also not mentioned a lot, but common to happen if you stop using the affected limbs. Many people compensate for the pain by using the limb less affected, which leads to muscle wastage, another reason they do encourage continued exercise.

I know a lot of people wonder about the effects on pregnancy, I won’t lie, I do not plan to ever be pregnant and carry a child so I have not researched this myself. But there is this particular study you can check out for more information on it based in Britain. There are also a lot of posts on the Lauren Currie Twilight foundation site that include TAK, I have not read any of these yet but plan to. I share some of my emotions and feelings about my treatment and diagnosis across my socials but this post from end of August 2020 has some specifics of what I was dealing with at the time and this video where I look back on myself from a year earlier is very emotional but also very raw and depicts the emotional struggles people go through when living with chronic conditions.

Check out this post right before I was diagnosed with TAK.

There is also a debate about whether TAK and Giant Cell Arteritis are the same thing or not. Not enough research has been done, and the only difference seems to be TAK has the ability to make limbs pulseless but otherwise both do very similar things and Giant Cell seems to be more likely in older women, where TAK is often younger. Sometimes both are just called Large Vessel Vasculitis for this reason.

I hope this has been helpful in teaching you about what Takayasu’s is and what I go through with it. I would really appreciate if you donated to my Ko-Fi as I do create this content for free and this is my lived experience, I am unable to work but I love to create helpful and educational content when I have the energy and capacity. This piece is over 3,500 words with numerous references as well to make learning and further research as accessible and easy as possible. I want to help empower other people in similar positions as myself, it was hard for me to go through this only a year after my Crohn’s diagnosis, but I had definitely learned some lessons from Crohn’s and applied it to TAK. Follow my other socials for more personal anecdotes, research, and advice. I post on Instagram, YouTube, Facebook, and Twitter. I also Stream on Twitch sometimes, feel free to follow and join in on live streams. Also consider sharing my piece to reach more people. Please check out my other pieces linked below as well.

~ Artie


14 thoughts on “Takayasu’s Arteritis: Rare diseases Day 2021

  1. Thank you for searching your story and for educating others on this rare disease. I’m sorry that you’ve had to deal with this, and hope that it eventually gets better.

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