PVNS: experience and causes
Posted , 91 users are following.
I recently got diagnosed with the fairly rare condition called Pigmented villonodular synovitis (PVNS) a disorder which affects joints. In my case, it was my knee joint, as is often the case.
I'm posting this to help others better identify and understand this condition, and also to explore any possible causes.
PVNS is very easily misdiagnosed by GP's. It was misdiagnosed twice in my case, by two different doctors.
Because it's more common in the 30's and 40's age group, and the symptoms are similar to arthritis, it can be misdiagnosed as the early onset of arthritis of the knee/joint in people who are in the upper end of this age bracket.
In the case of the PVNS affecting the knee joint which results in a tumour growth at the back of the knee (as in my own situation), because the condition often arises after a trauma to the knee joint, the condition can be misdiagnosed as a simple lingering knee injury, leaking joint fluid, giving rise to a Bakers Cyst.
The symptoms of PVNS are both sporadic and very similar to both an ordinary knee injury and arthritis as the condition progresses. The joint becomes stiff and movement restricted. It's usually painless in the early stages but as it progresses it causes mild to moderate discomfort with a persistent burning pain around the joint area.
If left untreated, PVNS in its advanced stages, can render the joint completely unusable, requiring a complete joint replacement.
Here's my own experience of PVNS:
I was taking Tramadol (a painkiller) for back pain and was heavily into exercise and was also carrying a heavy bicycle every day up and down stairs. I injured my knee. This was due to the Tramadol I believe. My pain tolerance threshold was at a much higher level, so the injury didn't fully register and I continued exercising and carrying the bicycle, further straining the already injured knee.
Two years later, the knee joint gradually became stiffer and a lump appeared at the back of the knee. It became so stiff I could no longer crouch or bend down to tie my shoe laces. I went to the doctor and was misdiagnosed as mentioned. The second GP ruled out arthritis from an x-ray and to confirm his diagnosis of a leaking joint and Bakers Cyst, he requested an ultrasound scan of the joint. The scan showed an overgrowth on the lining of the knee joint and a tumour growth, resulting in a PVNS diagnosis by an orthopedic consultant.
A proof-positive diagnosis is carried out from sample by examining cells under the microscope from the joint which have the typical PVNS-like abnormal characteristic.
There's two forms of PVNS, localised and diffused. The localised version is what I have. It is a lot easier to treat using key-hole surgery with a better prognosis for recovery. Diffused PVNS affects the entire joint lining, requiring open surgery to remove all of the knee lining and it has a much higher risk (almost 50%) of returning, and requiring extensive physiotherapy.
PVNS is quite a rare condition as I mentioned. Therefore it's not encountered very much by GP's and there hasn't been much research done into the causes of the condition or why it returns in many sufferers. It's not thought to be hereditary, and as I mentioned, trauma to the affected joint is a common feature.
This leads me to wonder if certain medicines, or activities, may cause the condition. As I mentioned, in my own case I was taking Tramadol (at a normal dose of 50mg once daily) for several years, both prior to, and after my knee injury.
I'd be interested to hear from anyone else out there who suffers with PVNS and their backgrounds, in particular:
Anyone who has developed PVNS while being on Tramadol or similar drugs?
Anyone who has developed PVNS after a sports or work related injury, which they ignored?
How much time elapsed from any initial injury to PVNS symptoms and diagnosis?
5 likes, 296 replies
shannon12189 morphix
Posted
My daughter was diagnosed last year. She has been on Sertaline for ~ 6 years. Just wondering if anyone on SSRI's had this in common. I have read that SSRI's have a blood clotting factor that needs to be considered. She had the surgery successfully, but we are suppossed to follow up with another MRI at 6 m or 1 yr. Not wanting to face the trauma again we have waited until the one year mark. However, her oncologist made up believe that if it returns the best course of action is a very expensive chemo drug. Does anyone have advise or experience with this that you know of? Thanks, S
cherrie33 morphix
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After 14 years of dealing with pain I thought came from a botched miniscus surgery, I have been diagnosed with PVNS. It was a relief to finally have a diagnosis for the pain I have been in for years, but now I want to get to the root of the problem and there are not many answers out there. I was just diagnosed yesterday so my research has just begun. If you have any helpful resources, I would greatly appreciate the information! What I have found is that PVNS has been linked to a condition where the body cannot propperly process fat. I have also read that PVNS is an autoimmune disease. I found this extremely interesting because over the past 5 years I have been plagued with a number of autoimmune diseases including non alchoholic fatty liver disease. This disease stems from the same issue as the article I found on PVNS. My 40's have proven to be a stressful time for me and I packed on a lot of extra weight. Everything I have read about auto immune diseases says they are a result of a poor diet. After watching the documentary "What the Health," I took control of things and began eating all organic, vegan, and very little processed foods. I feel better, the weight is comming off, but it's still to early to know if I can reverse these issues with a healthy diet. Anyhow, I thought I'd share the interesting details of my case and will continue my research on the links between autoimmune disease, and the bodys inabilaty to propperly process fat.
helen30480 morphix
Posted
Hi, I've just read your post and I wondered how you are getting on post op.
I damaged my knee about 6-8 month ago and have just had an MRI that shows overgrown villus synovium. I'm awating blood tests and a Rheumatologist appointment and final diagnosis but I think I've got what you've had. If so, I'm very apprehensive about synovectomy (many years ago I was a Physio). So, how is your knee now? - thanks, H
amy35459 morphix
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My husband has PVNS in his hip, it has caused him so much pain. He's out of work. What did you ultimately do for yours?
Oldfatguy1 amy35459
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Ill toss out a comment here as well if you don't mind the intrusion. How long has you husband been battling pvns and what was his original onset? Mine was in the most common joint, the knee. It came on suddenly and violently with unbelievable bleeding. At noon it was fine...by 3 the joint had increased in size by over 4 ". The bleeding moved upwards and finally covered the entire upper leg into the groin and lower abdomen. This started 15 years ago last week. Before the end of the year I had 3 surgeries to open the joint and drain the blood, each time they removed around 200 cc. I also had either 5 or 6 draining by needle removing 65 to 75 cc. Then came the biggest mistake of all, 30 rounds of radiation and finally a year later the tkr. I was told by the ortho oncologist that one small bundle couldn't be touched as it sat in the middle of a nerve bundle and any attempt to remove it could result the loss of leg. 9 years later, due to some type of minor activities, the stem of the prosthesis broke loose causing it to move and hit nerves every time I moved my leg. The pain was unbearable. A revision was mandatory and the surgeon took the prosthesis out with his fingertips and found, the tumor had eaten up the bone causing the problem. The day after my revision I fell and broke the femur requiring another surgery and a hip to knee plate. When this was removed I wound up with staph twice finally having to undergo antibiotic infusion after having the prosthesis removed and going without a joint for 4 months. 11 surgeries starting at age 65 and I'm now 80 knowing I still have pvns. I have been going to a University medical center with a teaching professor as my surgeon. He continues to prescribe opiates as pain killers because he knows my condition. Unfortunately, when this 1st occurred I was going to an ortho group of 8 surgeons with ovwer100 years experience and no one had ever seen pvns. My Dr has seen closee to 3dozen cases with me being his oldest patient with the tumor.
That's my story and I can assure you it is a bumpy ride. I was told I was a 1 in close to 2 million. I don't know where you are located but I'm in the middle of the US. I've been told that Stanford university med center is one of the only med schools doing much meaningful research.
I don't mean to paint such a gloomy picture but this isn't a pretty disease. I Know a pediatric ortho at a children's hospital that had a girl with pvns and it jumped from joint to joint and she had 13 synovetomies by age 16.
Good lunch to you and your husband. This isn't a walk in the park.
amy35459 Oldfatguy1
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AndreTaylor morphix
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I was just diagnosed with PVNS in my left knee at 25 years old. It started with me going crazy on an exercise bike doing 40-60mph for 30 mins. I would begin to feel lateral knee pain but if I pushed through it, it would go away. Being the athletic no pain no gain guy, I kept on pushing through it for a week straight until I couldn’t anymore. So then I stupidly decided maybe some leg muscle work would help so my legs could handle the pressure I put on them. I worked my legs so hard that session that they hurt just from touch for a week straight. And im not just talking about the soreness pain, but almost a burning pain that had me wreathing around from a simple touch. Needless to say, I had probably caused some trauma to the knee.
After about 9 months I finally went to an orthopedic sports surgeon who ordered an MRI and found a benign looking lump. Sent me to get another MRI with contrast this time and onto an oncologist, who confirmed I had PVNS. He said I’ll likely need a joint replacement surgery when I get to his age and I have 3 options: 1) Surgery, 2) Radiation Therapy, or 3) Both. When I asked him what he would do, he said he would go with both.
Unfortunately at the time I had no idea what the heck PVNS was so I didn’t ask him if I had localized or diffused so maybe you guys can help? Now I’m on a cruise he encouraged me to go on and as amazing as this is, im finding it hard to enjoy due to the pain and anxiety/worry even with the prednisone I’m on.
So he mentioned there was a tumor/lesion towards the front lateral area of the knee that is on the knee and goes slightly up to the lower leg. I have swelling all around in my joint and right above the knee cap. Does this mean it’s localized because there is only one lesion? Or does this mean its diffused because the swelling is all over?
Also, what type of treatment would you guys recommend? Would it be better to go with both options as he suggested? Exercise is my passion and losing that would be very hard on me so I’m trying to figure out the best way I can go to ensure this disease doesn’t plague me for the rest of my life. Thank you for any help and support you guys can give. I really need it..
AndreTaylor
Posted
Btw would it be better to start a new discussion on this to get more people to read or is here fine? Sorry I’m completely new to this site. Thanks!
Roach AndreTaylor
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Hi Andre,
Sorry to hear you've developed PVNS. I fought the diffuse form from age 24. to age 50. If it were me - knowing what I now know - I'd definitely have surgery first, wait for the pathology report, and then have radiation if it turns out to be the diffuse form. If its just the nodular form, then the one operation should do the job. If its diffuse, you'll need to continue attacking & cleaning out the joint space regularly until the b*****d disappears. Mine was extremely aggressive, and unfortunately needed one or two ops per year, totalling in excess of 30 over the years.
I had a Yttrium synovectomy in 94 - which didn't work, but it did age my cartilage. And being a staunch & unrelenting 'Life, be in it' type, I wore out the weakened knee cartilage, and then destroyed joint beyond being able to have a normal knee replacement.
So in 2014, I had what's called a 'Distal Femur implant' done. Essentially, the entire knee joint from above the knee, to the top of the Tibia has been excised & replaced with a metal prosthesis. Good news is I'm living a better quality of life than I had for decades. Regularly doing in excess of 30k steps per day without pain. And by working on it, I have a range of motion that exceeds what people normally get with a standard knee replacement.
All that being said, there's no reason to think you won't beat this annoying disease. I have a great friend in Perth, West Australia - whom I met on-line as we both were looking for answers on this disease. She had a fairly aggressive case of the diffuse form. With multiple clean-outs, her PVNS subsided and she's been fine for almost 10 years now.
The only sad thing for me is that I'm now 53, and from 47 I wasn't able to obtain work. Now that I'm good to go again, I have now recent work history, and my age is against me. So I'm pretty much chronically unemployed. I still do a Catalogue run, however, in order to keep strengthening & building my knee, and my capacity - trying to walk without any slight noticeable limp. =D
Good luck! (y)
Oldfatguy1 AndreTaylor
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Andre....you ain't gunna 'preciate this but here goes......BTW if morphix is still on here, you'll here from him as well. For an almost unheard of ailment (1 in almost 2 million) this is brutal. I was 65 when my knee suddenly blew up. Within 3 hours it was almost 4.5 inches bigger than the other and internal bleeding was obvious (within a couple weeks I was bleeding back into my lower abdomen and groin). A couple weeks later the started scoping me and taking out soda can amts of blood and also using a needle. Over 1000 cc in 2.5 months. My ortho had never seen the problem nor had any of the other 7 guys so I made the rounds of hemotologists, rheumotologists and ortho oncologists. Finally the pvns was diagnosed and radiation was used. In retrospect, I wish I had never done that as it burned the he'll out of my leg but didn't get all of the diffusion so 8 months later I had a synovectomy done by the ortho oncologist at the university medical hospital and medical school and that got most of it but there is a little in a bundle of nerves and to get it all might cause loss of leg. A year later they finally had to do the tkr. Fast forward 9 years, the stem of the prosthesis broke loose and this time I went to the ortho oncologist again and was referred a bright young sports medicine Dr and associate professor. He took one glance at the rays and said the pvns had eaten the bone an weakened it so bad the stem had broken loose and was moving inside the bone and of course every time it moved, it touched a nerve setting me in a screaming mode. I've had lots of other problems with the leg but they are due to a break and 2 staph infections. I'm now almost 81 and still fighting the fight.
You didn't mention where you are located but if you are in the US there are a couple of medical schools doing research, one is Stanford. This isn't an easy or short term deal I'm afraid. Hate like hell to scare people on such an impersonal forum but there really isn't a better way, I guess. I really wish I had of done the synovectomy without the radiation. I think I would of healed better and faster after the tkr. Get all the professional advice you can before letting them do anything. There will be pain andcaggravation no matter what. Be sure and ask you surgeon how man cases He has seen and treated. If. He isn't at least fairly well exposed to the problem, look for someone else. My surgeon has seen 2 to 3 dozen because the serves much of 2 states. One other thing. Back off the exercise as it will only lead to pain.
fadi_48579 AndreTaylor
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Andre Email me. I'm 25 yr old and your story sounds exactly like mine. I would be happy to give you my advice and experience. I type 3 long summarys on here but it keeps getting deleted for some reason. If you do decide I will be happy to talk to you. God bless
Moderator comment: I have removed the email address as we do not publish these in the forums. If users wish to exchange contact details please use the Private Message service.
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fadi_48579 AndreTaylor
Posted
Going to radiation only stick to surgery I was in the same exact boat as you. I've tried posting two comments but for some reason they're not allowing me to so if you want privately message me and I will tell you my experience and will give each other our emails
fadi_48579
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Don't do radiation*
AndreTaylor Roach
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Thanks for getting back so fast!
So it seems like the general consensus is surgery first and then radiation if needed. So there’s no way to tell whether it’s the diffused or localized form without cutting me open? I am beginning to think I definitely have the diffused because I’ve heard the diffused is way more common in large joints like the knee (lucky us PVNS sufferers who get a rare joint disease but it’s more common to get the diffused form
). I also have so much swelling all over which I assume is the synovial fluid cells producing excess fluid all over. Maybe that means it’s diffused too?
30 surgeries! Wow I am so sorry you’ve had to go through that. This sucks that it’s so rare that there isn’t much research being carried out. I’m finding plenty of research studies being carried out that say drugs have worked for treating this condition, yet this isn’t a priority. It sucks for us.
I’m worried about going through what you had to. Running and weightlifting and stuff is my passion. Losing that would kill me. I’m happy the knee replacement is working out for you. It sucks having to go through that but I’m glad you at least have a better life now.
Your friend in Australia, did she do surgery first and then later get radiation? Btw what do you mean by clean outs? Is that just surgery that cleans out the swelling and bad cells?
I’m sorry your whole life has gone by dealing with this condition. I’m scared that will be me too. I’ve already left my job and have been out of work/school for 9 months. I can’t walk for more than 10 minutes if I’m not on prednisone. I can’t do any exercise. I’m in pain constantly so I can never enjoy any moment of life with the people around me. Life just sucks right now. Anyways, thanks for taking the time to reply. I wish you the best of luck and a long happy life.
AndreTaylor Oldfatguy1
Posted
Hey oldfatguy,
First of all, wow, I’m so sorry to hear what you had to go through. This is freaking crazy. Our quality of life is completely decreased. I have so many dreams and ambitions and even motivation, but now it seems like I’ll never be able to reach them if I’m constantly fighting this affliction. I’m trying hard to stay positive because I don’t even know if I have local or diffuse or how things will end up for me, but I’m just not hearing anything good. At first I thought maybe I have ITBS, then when that didn’t go away bursitis, after seeing the doctor I thought hey steroid shot and I’m good to go, then I get told there’s a lump, then I get told it’s pvns, now I’m thinking it’s likely diffuse and I’ll be fighting this for the rest of my life. It’s driving me crazy that I just can’t get a win. Now I’m hoping for a simple arthroscope surgery but the way things are going it’ll likely be open surgery then radiation then it coming back and who knows what else.
I hate that this has plagued your life. I hate that all that could go wrong did end up going wrong with you. Its at least not cancer (like so many people keep mentioning; it’s getting annoying ), but damn it’s a pain in the ass. I feel like at 25 I was just getting ready to set my life up for the future but now I can’t do s**t.
The strange this is I never had any problems until I started going hard on an exercise bike day after day and even pushing through the pain. When I was doing squat and stuff before, I was fine. But it wasn’t until doing the bike and then weightlifting after that that really set it off. Yours just randomly happened one day? No history of trauma?
Yeah I’m all done with exercise unfortunately. I can barely walk so exercise is definitely out of the question. I live in SC, USA. Not sure if there’s much going on around here studying this. I’m also uninsured and jobless due to this condition and have spent most of my emergency funds on seeing the 5-6 different doctors until I was diagnosed with PVNS. I’m not sure how much more I’ll be able to afford to do.
Thanks for taking the time to reply. I wish I could do something for you. To be 81 and still dealing with everything from this condition makes me really feel for you. You’re definitely a fighter. Wish me well and pray for me oldfatguy.
AndreTaylor fadi_48579
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Roach AndreTaylor
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Hi'ya Andre,
Yeah thanks mate, it has been a tough ride, but it makes you tougher too in the end. You know, pressure makes diamonds etc. =D
I'm sure your Orthopaedic specialist can confirm the type via an MRI scan, but you'd still need any tumour removed anyway. So they'll definitely be able to confirm which form you have once they open your knee and do the pathology. With the diffuse form - meaning that it's not just confined to a single nodule, but everywhere - the only effective controlling treatment is to surgically strip out the synovial lining of the joint - synovectomy - which is where the PVNS thrives. I had this done arthroscopically the first 5 to 6 times, but an arthroscopic synovectomy just isn't as thorough as an open synovectomy, hence the PVNS grew back quickly. So from there on for the next 25 or so, it was an open posterior/anterior synovectomy, once - sometimes twice - per year.
In my case, the disease was still present in the joint even when I had the whole joint removed for a distal femur prosthesis. No decent surgeon should consider doing a standard Knee replacement if you have active PVNS, because the disease will mess up a replacement by invading & weakening the union between metal and bone.
I think, as long as you don't let them give you any Yttrium injections you can beat this thing by working hard and staying on top of it with surgery until it goes away. I had 20 good years, water skiing, snowboarding, playing sport etc, even though I was having the surgeries. I now know it was the Yttrium radiation injection and my unrelenting activity level that caused the premature destruction of my joint.
The thought of having the surgery was just a minor inconvenience after a while - even though they're big ops. I used to see it as an enforced 3 week holiday, and took on the challenge of recovering as quick as I could to resume work. You get good at it pretty quickly. I'd do cycling, swimming, walking, stretching, massaging and do all of the right things in the early post op stage. By the time I had the biggest op - to remove & implant the prosthesis - I was so well equipped that I wasn't at all bothered & bounced back from that one lightening quick too.
My friend in Perth, had diffuse PVNS too. She had multiple surgeries first, and then radiation - Yttrium injection. Michelle wasn't half as gung-ho and physically active as I was, and her knee is still in great condition today, with plenty of articular cartilage. Yttrium was considered 'cutting edge' back in the 90s, but its a proven failure, so don't let anyone talk you into it. Beamed radiation is the go these days - if you need it.
I can appreciate your situation completely, and do empathise with you. You're kind of at the age where I met Mr. PVNS too. Hold fast Andre, and feel free to chat anytime. Look me up on FB, friend me and you'll see an album about my fight with PVNS.
Cheers for now mate, I'm off to delivery more catalogues now!! =D
Roger
fadi_48579
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Oldfatguy1 AndreTaylor
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I'm in a suburb of Kansas city and have been using the university of Kansas medical center but they don't ha e any real research just some surgeons that have seen a number of cases.
You never know what you are faced with in life. About 6 years into my p r problems my wife was diagnosed with Parkinson's disease. She has gone downhill so rapidly that she is now completely dependent on me for about everything. I have been fighting the final stages of her situation, assisted living but her dementia, depression and anxiety are so bad its to a point of necessity. I was hoping to keep her home till after the holidays but with the shape my leg is in its getting more and more difficult caring for her. I have to do what's best for both of us and assisted living may have to be the answer. She is alert enough to fight it but the kids are pushing her in that direction.
Keep fighting, you can't give up. Keep us advised