PVNS: experience and causes

If PVNS is rare then my brother in law is special because he got his in the mandibular joint hahahahha. Relax, I am not unsensitive, I am happy because the doctors thought is cancer so duh, I'll take PVNS over cancer, don't you think? He is now being shopped around for surgery as it seems doctors not only have a hard time identifying the problem but are also lazy or scared, very polite, very concerned but if you really need them...oops, they don't do that. I don't know exactly what they do besides writing prescriptions...I'll digest what you guys write here, after all we can't depend on doctors to learn or cure anything, we have to do it ourselves for our loved ones, right? Right.

From many discussions on the Facebook support page "PVNS is Pants" and from doctors who have some experience with PVNS in the US I think that this disorder is definitely something we are born with and it takes it's time growing at first which is why many of us make it to our 30s to 40s before a diagnosis. If you sustain an injury it can trigger its growth at a much more rapid rate. Also there is discussion of a gene known as MTHRFR (Methylenetetrahydrofolate reductase) being found in those of us that have PVNS. This could be the cause but who really knows? Have yet to find a team of doctors dedicated to researching this although there are many abstracts that Doctors have posted on the internet, most of them are just guessing at this point. I found this link to be the most helpful with describing what the doctors are dealing with:

http://www.pathologyoutlines.com/topic/jointsPVNS.html.

However, most doctors in the world are not familiar with PVNS and either won't touch it or will misdiagnose it. The doctors who give any kind of time line or numbers associated with rate of growth, rate of relapse, or any other rates without subsequent proof are liars. Seems to be quite a few of them making bold statements to keep our hopes up. For anyone looking for answers and being newly diagnosed, PVNS sufferers face potential annual surgeries until all PVNS cells are fully eradicated, but that doesn't mean it can't show up in another joint. That's my take on it at this point. I've been suffering knee issues since 1987 and was only diagnosed last year in 2013 at the age of 40 after I insisted that someone figure out why I could no longer walk. 7 doctors later, I got my answer after 2 sets of xrays (which don't diagnose PVNS at all) and an MRI with ink injection (that was the only way they were able to detect it).

Hi georgechi , had similar problems with hospitals near me. I highly recommend the Royal National Orthorpedic Hospital in Stanmore London. Specifically Mr Pollock.

Alice thanks, I did a little flyover them and are definitely on the list. We are in Chicago though and the guy is crazy, driving his wife crazy, driving my wife crazy, wants to talk but doesn't want to talk, blames no one ut somehow blames everyone else, is hard to make plans with scared people. who don't understand they are hard on us for no reason, we don't have any answers hahahahha. Anyway, I'm checking out New York and LA in addition to Chicago and if everything in US fails..is UK baby I don't care and neither should he, after all we should use any chance we have to go on vacations, even medical ones, right?

My daughter is 12 years old. She has had knee pain and swelling in the back of her knee for a year now. She is a basketball and volleyball player so we assumed it was a bursa cyst and we managed it with anti inflammatory meds and icing and wrapping. It continued to hurt so we took her to an orthopedic surgeon who immediately ordered an MRI. We had a knee scope two days later and she had 2 small masses of PVNS. It is now the day after surgery and we are waiting for pathology reports but it visually looked like pvns. I guess after reading this forum I need to ask if it is localized or diffused but where it was 2 tight balls I am assuming it was localized. What can I expect for her future? She is extremely active and I would hate for her not to be able to play the sports she loves.

Hello

It's nice to see so much discussion on this rare disease so I thought it would be a good place to share my experience with PVNS.

When I was young I was pretty athletic and did a lot of activities that involved repetitive motion in my ankles, e.g. skateboarding and drumming. One day when I was 15 years old I had a slight pain in my right ankle that started to get progressively worse as the day went on. I had overextended the ankle a few times before and always walked it off, so initially I thought it was due to that, but I hadn't injured it for weeks prior. That night the pain and swelling climaxed in a dramatic fashion, literally the most painful experience I've ever had, randomly out of nowhere. We went to the doctor the next morning after a sleepless night and the pain and swelling had somewhat subsided. They did an x-ray and couldn't see anything. All they see is a kid with a swollen ankle, so they initially write it off as an injury, which I couldn't really refute since I had mildly sprained it the summer before. This was the first instance of negligent misdiagnosis that would lead me to endure this disease untreated for the next 11 years. Of course the symptoms came back. The pain and swelling came in transient episodes that tended to revolve around atmospheric pressure changes, classic signs of synovitis of any form, yet they were persistent in misdiagnosing it and forcing me to go through unnecessary medications, appointments, and procedures for gout, pseudogout, or bone spurs. I stopped going to doctors for this condition after they unsuccessfully attempted a fluid tap of the ankle and got nothing. I was so frustrated and in hindsight, It takes a pretty stupid doctor to attempt to blindly stick a large needle into an unknown mass under the skin without doing an MRI. Over the next three years I lived with it and moved to a new town to go to grad school. The symptoms progressed and started really impacting my everyday walking. Luckily the town I moved to has the largest medical center in the country. I go in to a GP and the first thing they want to do is check for blood clots because all they see is a swollen ankle. Great, more beating around the bush. I highly suspect I would have gone through another series of doctors that would have attempted to treat the symptoms by not actually looking at the affected ankle had I not specifically told the GP to refer me to an orthopedic specialist to consult for exploratory surgery. I went to the orthopedic specialist and we did an x-ray on the spot. Something new had developed in the last 3 years, a dark spot (lower bone density) in my tibiotalar joint, and some bone erosion in my fibula. They ordered an MRI (finally) and it revealed a severe case of diffuse PVNS that had proliferated through the entire cavity of my tibiotalar joint. The swelling of my ankle wasn't fluid at all and the protrusion on my anterior ankle, about the size of a golf ball by this time, was just the tip of the iceberg. Due to the synovitis, my body's immune system had eroded part of the fibula and also caused large bone legions to form in the tibia and talus which is where most of the discomfort in walking stems from. I can't help but feel like if something had been done earlier I could have avoided the irreparable damage that's been done to my ankle, but then again PVNS could have been treated and come back 3 times over in an 11 year span. The treatment at this point was clear: full synovectomy. The procedure required open surgery with two 5 inch incisions on the anterior and posterior side of the tibiotalar joint. The surgery displaced a significant amount of articular cartilage so I will likely have more symptoms of arthritis later in life. That was a month ago. I'm recovering slowly and can put weight on the ankle now, but it will be difficult to use for the rest of my life. I'm hoping physical therapy will help me regain at least as much use of the ankle as I had before surgery. If the PVNS returns, I don't know what I'll do. I just hope it proliferates as slowly as it did before. It's important to be proactive with health conditions and to be assertive with doctors who are giving non specific diagnoses. It seems I learned that the hard way. In the future, I may have to have an ankle replacement. I would be interested in discussing this with someone who's gone through a joint replacement as a result of PVNS.

Hello, I was diagnosed with PVNS in my left knee nearly 34 years ago. For many years, the pain and

swelling were tolerable; eventually, PVNS destroyed the entire joint and I had a total knee replacement. I

think that my initial problem began when I fell on an escalator in 1976; the disease took a while to really

become problematic. Prior to the total knee replacement, I had two arthroscopic surgeries; both times, the

symptoms returned within months.

Hello,

I'm 51 year old man in USA. I was having much pain in my knee for years. I went to several doctors over the years and they just keep saying it was nothing serious as they couldn't find anything wrong. Then in 2005 after I fell down and had to go to the hospital with injuries to my head the doctors started to look more closely at my knee. They did a MRI but the only thing they found was a torn Misniskis in my knee. The surgen said that wouldn't stop my knee from failing but he could use that as a way preform an Orthoscopic of my knee. That is when he found I had localized PVNS.

He removed 4 tumors and said the chance of it coming back was very remote. From 2006 until summer of 2009 I was fine. Then in the summer of 2009 the PVNS was back. In Jan 2010 I had the Ortho again and this time it was defused. Six months later it was back and the doctor sent me to Stanford Medical Center where they have specialist for tumors. The chief doctor there at first didn't think I had PVNS as it is very uncommon. They preformed an Ortho of my knee and confirmed I did have PVNS and this was the third time. After removing the PVNS again they had me take a low dose of Chemo for 60 days. That still did not work and in February 2011 I had to have my forth Ortho to remove the PVNS again. After that, they did Radiation on my knee for 2 minutes per day for 17 days. The PVNS still came back, this time the doctor at Stanford said there was nothing else he could do for me. As a disabled veteran I went to the VA and asked them to see if they could help. They just completed my fifth Ortho on my knee. The doctors there say if it does not work this time then they will do a full knee replacememnt.

I have had the same problems with my other knee but as of now I still have not had anything done to it. The decision by myself and the doctors is to wait until the right knee is completed either with no signs of PVNS or a knee replacement then we will look at the left knee to see if the PVNS is in there.

After having five Ortho surgeries on my right knee, I can tell you that MRI's do not always pick up the PVNS. In my case, I have had five separate occurrences of PVNS and it only showed up three times on the MRI.

The longer you have PVNS the more persistent it becomes and the more painful it is. There have been times that just trying to slide my pants on over my knee causes great pain. Now that I have had the PVNS so long the tendons in the back of my knee have been torn up and there is lots of snapping and looseness in my knee. I have to wear a knee brace for now while we wait to see if the PVNS comes back. The doctors don't want to fix the tendons until we have confirmed that the PVNS is gone.

Over all, PVNS is a nuisance and painfull condition. I wish there was more research that was done on PVNS and see what the comon denominator the people that have it have in common this way maybe the doctors could diagnose it more easily and others could get it taken care of much sooner.

Hi all, I'm Paul, the original creator of this thread (for some reason the site lost my login profile and changed my username to 28289201!).

Anyway, for those interested.. I have received bad news.. It's been 8 months since I had my surgery to treat and remove PVNS affected knee tissue.

At first, all was great and I thought I was in the lucky 50% that was cured. I even managed to climb the Rock of Gibraltar last December without much discomfort and thought I had my life back on track.

However, over the last few weeks the disease has returned and begun manifesting itself in the usual dull pain and stiffness, especially when doing any lengthy walking or anything physically demanding.

So it seems I'm not in the lucky 50%. I went back to the hospital today to see the Dr and they examined my knee and said the disease is not gone, but just slowed down, that's what the treatment was for apparently (I was not explained this). They said some patients go on to ok and only have minor troublesome return of symptoms, but others have the disease get progressively worse and require more surgery.

The Dr said my leg still had good movement and the pain wasn't sufficient to warrant further surgery at this stage (or even scanning to check its progression) due to the risks and practicailities of 3-4 months of recovery time, so soon after surgery last year.

She said I'm to return in June for a scan, and they will assess the situation then. I feel they're trying to keep me positive and holding back on me, but I can tell from what I know and the Dr's face that it's not good, and I'm likely to face further and probably regular surgery in the coming years.

At the moment I'm taking a painkiller daily to mask the dull ache which is slowly returning and nearly ever present, but my leg is not stiff as was before, so no stick.. so I'm counting my blessings!

Hey out there, been following this forum for a while now as I have been one of the few PVNS folks.

Quick history, 52 yrs old and was a sports fanatic since childhood and an avid competetive cyclist for over 15 yrs.

Back when I was 17, I had tore the miniscus and ACL and at the time, they didn't have orthoscopic surgery yet and they took out 80% of the miniscus and I had elected NOT to get the ACL repaired.

So, when I was having sever pain and swelling in my knee approx 3 yrs ago, they did xrays and MRI which showed that I had severe bone on bone but nothing even came up about this think called PVNS. Guessing we all thought that all the symptoms were synonimous with severe artheritis. the Orthopedic surgeon did NOT want to perform knee replacement surgery due to my age so we held off for a while until it got to the point I was begging to have it done.

During the replacement surgery 9/2012, the Dr was familiar with PVNS (thank god) but had never personally seen it before. After scraping and cleaning out as much as he could find prior to putting in the new knee (normal surgery ~3 hrs, mine ~6.5 hrs with a ton of blood loss).

My rehab has been a roller coaster. As I'm a no pain no gain guy. I seemed to have been too aggressive and after making some progress ever couple of months of rehab, I would increase the intensity of the exercises and my knee would blow up like a ballon. This went on for a full year or 5 setbacks…

After putting up with this for a year, in 9/2013, I had decided to take it really easy and let the knee heal internally for several months before jumping back into more exercises to help strengthen the now severly atrophied muscles.

Well guess what, as soon as I started increasing the intensity, my know blew up like a balloon again and this time my lymph nodes on my left pelvis area (same side as PVNS knee) blew up like balloons and now have a rash that won't seem to go away.

Unfortunately, Dr has been unable to find a Dr in Houston, TX that has had any experience with PVNS after a full knee replacement so he is flying by the seat of his pants.

He is baffled and concerned that there may be an infection in the leg or bone -or- a loosening prosthetic -or- PVNS recurrence - or- no clue at all. So had a CT scan, bone density test, and blood pulled to run a multitude of tests to see what is ring on in there…

Thus 1.5 yrs later, back to doing little of anything until Dr gets these results and we can determine next step.

If anyone has had a total knee replacement due to PVNS, I would certainly like to hear how you are doing and what you did to get back in the normal life swing of things…

Thanks again and look forward to all feedback.

Hi Craig, this is a great forum and I have learned so much from other people's experiences. You may want to also post your story and questions on "PVNS is pants" on Facebook. It is a large page and there will be many people on there who live with PVNS after joint replacement and even revisions on joint replacement. You may have to travel to find the right surgeon. I live in Los Angeles and my husband has PVNS in his knee. We consulted with orthopedic oncologists (musculoskeletal tumor surgeons). That type of surgeon would also be able to work with your knee replacement. My husband is not at that point yet, but I am an endurance triathlete (just a finisher) and I can only imagine how you must feel. What an awful journey for you so far. Don't give up. There is a lot of help and support out there! Best of luck to you and let us know how things progress.

hi Craig

I had a total synovectomy ( not a scrape ) and knee replacement 8 months ago. The PVNS was not diagnosed before the surgery. It was only the second time my surgeon had seen PVNS. Recovery has been slow and although I can bend now to 95, I still cannot straighten my knee fully. The swelling is expected to be with me for a year at least.

I was 69 at the time of the surgery which is old for PVNS. You are the first person I have found on several global websites to have the same as me. How old are you? I am still trying to find out how long before I can be back to tennis. There is no case history to help. I have had regular physio sessions at the hospital and also joined a gym to exercise the knee and quadriceps, which are easily wasted with lack of exercise. I still have pain and stiffness and find it hard to walk down stairs freely. Hope this helps.

Thanks Urs & Peter for your feedback.

I will check out the Facebook forum and see where it leads…

I'm 52 and had the replacement at 51 (replacement ~1.5 yrs ago). Dr said the recovery would take quite a bit longer than someone who had not had PVNS as there is quite a bit of muscle and tendon trauma from the synovectomy, so I need to be a bit more patient (which is not one of my strong suits).

Hang in there Peter, took me a while to get any sort of range of motion and was really excited when I was able to hit the 90 deg mark and make a full circle on the bicycle pedal stroke. Fortunately, getting flat wasn't an issue for me, but took me at least 6 months to get past the 90 deg mark with a bit of force… Fortunately, stretching has been my friend and took me about 9 months got to ~110 deg and now hitting 125/130 deg (with effort).

Best advice I had received from everyone I know (of which, have never heard of PVNS) is to take it easy for a few months. That really helped as all I did was stretch, stretch, stretch. The good new was my range of motion really started to accelerate - Bad news, my muscles attrophied even further so had a bit of catching up to do which caused much discomfort and being extremely sore ALL the time. But eventually, at ~15 months got some of the muscle mass and strength back and my gate is getting pretty close to normal with strides being of equal length and duration.

After the tests earlier last week, I had been taking it really easy doing nothing but the day to day walking to and from the car to the office, stretching every morning and evening, and icing every night, the swelling has reduced a bit.

Took a flying leap yesterday (Sat) and did a 30 min ride on the trainer with cadence of ~80-85 in low resistance followed by a 1/2 mile walk then stretched and iced. Swelling seem to have been kept at bay so I did the same this morning (Sun) and in the icing process now.

The Dr should have all the test results tomorrow (Mon) and hoping to see what has caused the rash, enlarged lymph nodes, and really low blood count.

Stay tuned….