I have a diagnosis of limited cutaneous systemic sclerosis, which was diagnosed about 3 years ago have been relatively ok until recently, when I started to develop problems with my oesophagus. I'm still waiting for the results of my barium swallow, which apparently can't be accessed currently because of the cyber attack.
I've also had some quite significant skin tightening on my neck, which has appeared quite quickly, mainly over the past week. I've never seen anyone with scleroderma though, and so I don't know if this a normal development of scleroderma or not. I've tried googling for images, but can't see anything that looks like my neck. It is the same colour as the rest of my skin, and is normal when I'm looking straight forward, but is really noticeable when I lift my head up, or look to either side. It then looks a bit like someone who has had a severe burn, and really hard (to the point that I can actually knock on it!). It's also sore, and is limiting my range of movement in my neck. I have a sensation of being strangled, and I think it's just because the skin is so tight.
I've tried ringing the rheumatologist, but the secretary said that she couldn't do anything because the computers are still down. I've therefore made a GP appointment for tomorrow, although I doubt they'll have any idea what it is!
Has anyone else experienced anything similar? I'm wondering whether this means that I have now developed diffuse cutaneous systemic sclerosis, but am quite frightened by the speed that this seems to be happening. I thought these things would take years to develop.
Any ideas would be very welcome! x
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LittleMissH
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I have limited systemic sclerosis. I have skin changes on my neck and chest Mine is very dry and tight with pigmentation change. I know it doesn't sound like your but why I'm saying it is because it's on my neck. I had a barium swallow which they say is normal although I have the worse reflux. Fingers crossed you can get to talk to someone today
Hi LittleMissH , skin involvement on the neck is consistent with the distribution of affected skin in diffuse systemic sclerosis, not limited. Do you know which individual scleroderma antibody you tested positive for? Have you seen a scleroderma specialist, as opposed to a general rheumatologist? Most general rheumatologists and primary care physicians have little, if any, direct experience with scleroderma and it's an extremely complex disease. If you have copies of your lab results and want to post them here, I can help you to interpret them. Your antibody type will be associated either with limited or diffuse, limited can't change into diffuse over time. Rapid skin changes are also a characteristic of diffuse SSc, I'm afraid. Are you on a DMARD or other drug treatment yet? Has your doctor ordered pulmonary function tests and an echocardiogram? These should be checked early and then at regular intervals in systemic sclerosis so any changes can be addressed as early as possible.
Sami13 , skin involvement on the neck and chest is characteristic of diffuse SSc, not limited, so I would ask you the same questions as above. Best of luck to you both, I hope you'll keep us posted on how you are doing.
I definitely have limited SS. I have anti centremere positive I'm under the royal free. Professor Denton. I'm on methotrexate. Doesn't seem to be helping much at the moment Fingers crossed
You're in very good hands, but it's very unusual to have skin involvement on the chest with centromere positive limited systemic sclerosis. Is the skin on your chest and neck characteristic of scleroderma changes? I'm just curious because in addition to antibody type, the defining designation of lcSSc is skin involvement "limited to" the face, legs and feet below the knees, and hands and arms below the elbows. When it extends to the trunk, it is considered to be a skin distribution consistent with diffuse. Interesting!
They didn't seem worried or surprised. I don't have skin changes on my face or legs. Just hands arms neck and chest. I also have the calcium growths on my lungs not on my joints which is unusual. Maybe I'm just odd !
We are all odd with this because the numbers of people are so low, it is hard to find statistical data on each individual type. Frustrating to have "orphan disease!"
I was thinking along the same lines, Diane, and wonder if there is some kind of mixed presentation possible. I have limited SSc, but I get weird adhesion-kind of restrictions in my upper arms as well as my lower arms, and have to have it worked out with physical therapist to break up the fascial "stickiness" and then do lots of exercises to keep it loosened up.
Makes me wonder if there are tiny subsets of non-traditional presenters but not enough data because these "weird things" don't make it into statistical significance.
We should all get together and do a qualitative study where we report all our "odd things"!
It's a very good idea. Just recently been diagnosed with diffuse systemic scleroderma and I'm more than happy and willing to join. Hopefully with more collective data there will more understanding to this disease.
I am just wrapping my my PhD, and when it is over I will see what I can pull together. I will see if I can use this forum to recruit people to share their experiences. Would be so cool to hear in people's own words what they are experiencing, and link it to the different types, etc.
Thanks for your reply dianekjs. You you sound very knowledgeable! I have a 'positive ANA centromere pattern', although I've no idea what that means!
I originally saw by my GP, who fortunately works in rheumatology once per week, and so was able to quickly diagnose me quite quickly. When he referred me to rheumatology I saw him again, but have since been transferred to one of the rheumatology consultants there. He's not a scleroderma specialist though. I'm seen in the monthly 'connective tissue disease clinic', although I'm not sure they have many patients with scleroderma.
I have has an echocardiogram, and I've also been sent for a lung function test, so they do seem to be doing the right sort of tests. I'm on Hydroxychlorequine, and also nifepidine to help with my raynauds.
I'd love to see a scleroderma specialist. Sami13, how did you get referred to Professor Denton? Is it just that you're local to the Royal Free? I've never been given an option!
The rheumatologist referred me. It sounds like you have all the right tests done not sure that the specialist would do anymore but it's always good to see them. You could ask at your next rheumatology appointment. Do you have any other conditions or symptoms. ? We sound very similar
We do sound quite similar! How long ago were you diagnosed?
My main problem so far is that I have quite severe Raynauds, and had ulcers on my fingers in the early days as a result. I'm just really careful about keeping warm now though, and so haven't had any ulcers recently. I also had quite a lot of reflux early on, but this sets stopped probably about a year ago. I also have puffy fingers and stiffness in my hands.
What is the methotrexate? Is that similar to Hydroxychlorequine, or something different?
I have bad raynauds too it's horrible had it since I was a child be it has got really bad the last few years. I have been getting reflux lately but the barium swallow was normal. I have really achy legs. I have so many random symptoms I feel like I make it up sometime! I'm on methotrexate now. And losartan for the raynauds. It doesn't seem to be working my raynauds has been awful lately. My circulation is my biggest problem I feel think that's part of some overlap connective tissue disease
I'm feeling for you with the raynauds! Our heating bills are sky high, and I'm still wearing gloves and using hand /foot warmers every day (even though it's nearly summer).
I know what you mean about random symptoms as well. I often don't think to mention things to my doctors, as it hasn't occurred to me that they are anything to do with my scleroderma (until I read it somewhere).
I have got masses to learn. My rheumatologist has not given me any information at all, and everything I know is from the Internet. This forum has taught me lots! x
Well, hopefully it will turn out that the centromere staining pattern is accurate and that your individual antibody is indeed anti-centromere, and therefore associated with limited SSc. However - ANA staining patterns are unreliable because their interpretation is subjective and can differ from one technician to another. So, to get around that issue, scleroderma specialists (and hopefully some rheumatologists) always follow up by ordering an anti-centromere individual antibody test for confirmation. That is the next step that needs to be taken.
The reason it is so important to get an evaluation from a true scleroderma specialist as early as possible in the course of the disease, is because other rheumatologists and primary care specialists simply don't have the experience with this disease that is necessary to optimally diagnose, treat, and manage it. It's just too rare, WAY too complex, for the non-specialists to stay on top of, and treatment options and recommendations are constantly evolving. You may have to travel for an initial consult and work-up, but most scleroderma specialists can then coordinate ongoing care with your local doctor. I'm in the U.S. so am not too knowledgeable about names of specialists in the UK, but I do know that Prof. Denton is recognized as a scleroderma expert there and have read enthusiastic testimonials of people who have been seen by him. I would imagine that you can request a referral, but your GP should order the anti-centromere antibody test first.
That's good that you have or are getting baseline testing for heart and lungs. Pulmonary function tests give the most information and don't use radiation so are very safe. Both the echo and PFTs are typically done annually in scleroderma patients.
Thank you Diane, I'll do that! I'm seeing the rheumatologist for an 'urgent review' sometime in the next couple of weeks. Will the results of the test change the treatment in any way?
By the way, I'm impressed with your vast knowledge! Do you work in a related field?
If the centromere antibody test is negative, additional testing will need to be done to determine which scleroderma antibody you have. The next most common antibodies to check for would be SCL-70 and RNA polymerase III. SSc antibodies are almost always mutually exclusive and there are approximately eight of them. You would only be positive for one. Each antibody is associated with different disease characteristics, risks, and prognosis. Once you have confirmed the antibody, it is possible treatment could change. For example, if you end up with a different antibody that is seen in diffuse, your doctor may want to be a bit more aggressive than hydroxychloroquine alone. Similarly, with either limited or diffuse, if internal organ involvement is noted or showing signs of progression, a stronger DMARD (disease-modifying anti-rheumatic drug) may be indicated. Let us know what you find out and how you are doing.
You could ask your rheumatologist to refer you to Professor Denton. My rheumatologist did it without me asking but I think he may have know him personally from college.
Professor Denton is in charge of research into Scleroderma and is one of the best in the world. You can read about him on this site and he has an article in the quarterly magazine you can subscribe to.
Not long after I was diagnosed I asked my GP for a refer all to Prof Denton at the Royal Free. I wasn't waiting too long before I got an appointment. It is quite a trek for me as I live in Yorkshire, but worth it to know I am getting the best care possible. I now go every 6 months as well as seeing a rheumatologist locally.
I think that's what I want to do too, and it's good to hear that you're able to go all the way from Yorkshire. I'm in Derbyshire, so not quite as far as you, but still a bit of a trek. I wasn't sure if they'd say no because of where I live.
Does your local rheumatologist communicate with Prof Denton at all? And are you able to have routine investigations at your local hospital rather than travelling to the Royal Free?
I'm due to see my rheumatologist in the next couple of weeks, so I'll ask if he'll refer me. Do you think my consultant will be amenable? I don't want them to think that i don't feel they're doing a good job, but I know that they can't possibly have the same level of experience at the Royal Derby Hospital.
I felt the same as you that my rheumatologist might not like me seeing another consultant but he was fine with it and is always interested to hear of any new developments. He acknowledges that prof Denton is one of the best on the country and knows far more than he does about scleroderma. I expect yours will be the same. It really is worth going to the royal free. They have access to the latest technology. Far more than my local hospital and they have a specialist nurse who will also give you advice.
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