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Has anyone had a late or slow onset of diffuse skin involvement?


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#1 greypilgrim256

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Posted 20 February 2015 - 08:46 AM

Is there anyone here that has had a late or "slow' onset of skin involvement typical of diffuse scleroderma? 

When I say late, I mean over a year+ from first symptoms. 

I had all the classic symptoms of diffuse sclero like the swollen hands, joint aches, a positive diffuse antibody, and tendon friction rubs, but I still have no developed wide spread skin thickening, nor do I have any evidence of thick skin above my elbows, knees, or on my trunk. 


Has anyone had initial symptoms develop and then YEARS later progress to widespread thickening?



#2 Amanda Thorpe

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Posted 20 February 2015 - 11:19 AM

Hello Greypilgrim
 
Interesting question but I think the answer is no. Part of the diagnosis of diffuse systemic scleroderma is rapid skin involvement. Part of the diagnosis of limited systemic scleroderma is slow skin involvement, as in can take months or years.
 
Typically limited systemic scleroderma starts with skin involvement on the hands, maybe forearms, feet, lower legs and the unwanted and unpleasant changes of the face and facial skin. Usually this involvement is mild, occurring over many years. Internal organs can be involved, as with diffuse, with gastrointestinal problems being common. Raynaud's Phenomenon usually precedes any skin changes, by months or even years with the eventual skin changes being mild. About 70% of people with systemic scleroderma have the limited form.
 
Typically diffuse systemic scleroderma starts with rapid skin involvement that can spread over the body within months. Skin changes affect the hands, forearms, feet, lower legs and also the upper arms, thighs and trunk. Internal organ involvement can also be rapid and life threatening, with immunosuppressant therapy commonly prescribed early on. Raynaud's Phenomenon can occur at the same time as the skin changes or one can quickly follow the other.
 
Both types of systemic scleroderma can affect the internal organs and can be life threatening so both need careful monitoring in the early years.

 
Having considered things I have to say that, no, I do not know of a diagnosis of diffuse systemic scleroderma with skin involvement coming on months or years later. By definition that would have to be a different disease surely? How could it be diffuse systemic scleroderma if rapid onset widespread skin involvement is not only a symptom of diffuse but one that differentiates between limited and diffuse.

 

What about either scleroderma sine scleroderma or scleroderma sans sclerodermaScleroderma without skin involvement. As per our resources:
 
"Sometimes Diffuse Systemic Scleroderma leaves the skin and joints untouched, and affects only the connective tissue of the digestive system or some other internal body system. In the absence of visible skin involvement, "Scleroderma sine Scleroderma," is diagnosed, which basically means "Diffuse Scleroderma without Scleroderma (skin involvement)."

In Scleroderma sans Scleroderma, Raynaud's may or may not be present. The usual presentation is with pulmonary (lung) fibrosis, and/or Scleroderma renal (kidney) crisis, and/or cardiac (heart) disease, and/or gastrointestinal disease. Antinuclear antibodies (ANA) may sometimes (but not necessarily) be present (Scl-70, ACA.)"

 

Remember that I am not medically trained and have simply told it as I understand it, I hope it helps. You might find my blog about this subject interesting, "Diffuse or limited, that is the question, scleroderma by any other name".

 

Take care.


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#3 Shelley Ensz

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Posted 22 February 2015 - 09:06 AM

Hi Greypilgrim,

 

I just wrote a nice long reply and lost it all by accidentally hitting the back button.  So this will be much shorter! 

 

As it happens, there's huge variation in all types of scleroderma; and I think Amanda is referring primarily to the rapid-onset form of severe diffuse scleroderma, which thankfully is the most rare because it carries the worst prognosis of all. But "regular" diffuse scleroderma can develop at any pace, although overall it tends to develop faster than Limited Systemic Scleroderma.

 

There's an old abstract by Steen and Medsger, Severe organ involvement in systemic sclerosis with diffuse scleroderma, which says, "Of the 953 patients with diffuse scleroderma, kidney involvement developed in 177 (19%), heart involvement in 143 (15%), lung involvement in 151 (16%), GI tract involvement in 74 (8%), and skin involvement in 233 (24%). Severe skin and kidney involvement occurred during the first 3 years in 70% of those who ever developed these problems throughout a mean of 10 years of followup. Severe heart, lung, and GI tract involvement developed during the first 3 years in 45-55% of those who were ever affected."

 

All of the emphasis is mine. Make of the statistics what you will, but basically, the first three years are the riskiest with diffuse scleroderma, however based on this study, having diffuse scleroderma would make it much more likely for you to not develop skin involvement over the next ten years!

 

That's the up side.  The down side is that, however unpleasant and even disabling it can be, scleroderma skin involvement has never killed anyone.  It is the internal organ involvement that can be life-threatening, and with diffuse scleroderma, kidney involvement is the greatest risk of all, but three is still a 4 out of 5 chance of escaping that particular problem...if you keep your focus on the sunny side of the statistics.

 

:emoticons-group-hug:


Warm Hugs,

Shelley Ensz
Founder and President
International Scleroderma Network (ISN)
Hotline and Donations: 1-800-564-7099

The most important thing in the world to know about scleroderma is sclero.org.

#4 greypilgrim256

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Posted 23 February 2015 - 05:22 AM

This is so strange.  I have a diffuse antibody (U3RNP), large joint pain, tendon friction rubs which are essentially a sure sign of diffuse skin involvement, but my skin changes are limited to my hands, ankle area, and now I feel my face being affected, but it is all very minimal changes.  

 

The 15mg methotrexate seems to be helping my painful tendon friction rubs and joints, but i still feel my face feeling "weird".  Hard to explain.  Don't get me wrong, I am VERY thankful I do not have serious widespread skin involvement, but I am very confused because all the signs seems to point to having diffuse.  

 

I was also under the impression that diffuse sclero moves FAST.  Who knows.  All I know that this disease is awful.  

 

Is it possible for the disease to "back off" after a while? 



#5 Shelley Ensz

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Posted 23 February 2015 - 09:51 AM

Hi GreyPilgrim,

 

I believe there was a study a few years back in the U.K. which showed that about 40% of systemic scleroderma patients don't even know exactly which type they have, presumably because our doctors never told us. Often that might be because even our doctors are not entirely certain.

 

It is not unusual for things to be fuzzy or borderline because this is not a cut-and-dried disease.  In fact, its more of a moving target, since researchers seem to be forever debating how to classify the subtypes.  One hazy thing that began years ago is that there are two different systems for systemic sclerosis.  One considers skin involvement above the wrist to indicate diffuse scleroderma; the other insists that only skin involvement above the elbows indicates diffuse.

 

So by this reckoning, someone with skin involvement only up to their elbows would have limited scleroderma according to one doctor, and diffuse scleroderma by another. Keep in mind, the *only* thing that limited versus diffuse scleroderma really means is that the skin involvement is less extensive in limited scleroderma. Either type can affect virtually any part of the body, or occur at any speed. The involvement can range from fairly mild to severe to life threatening, and such complications can set in at any time over the course of the disease.

 

Also, any type of systemic scleroderma can spontaneously settle down, or even recede, even without any treatment. In fact, the normal course of the disease is to wax and wane, and that is what makes studying treatments for scleroderma invariably confusing and frustrating for researchers.  It is common for them to think they are on the brink of a cure for scleroderma until large-scale clinical trails are unblinded and reveal that the drastic improvements that were seen were just as common in both the treated and the placebo groups.

 

Since there is a such a strong placebo effect in scleroderma, so strong that it even confounds the researchers, one of the important take aways is that it could be very helpful to all of us to *believe* that our treatment plan or health habits will improve our health.  A positive attitude is very likely a boon for us, which is great news as being positive is only a learnable skill and it is not dependent on our circumstances or genetics.

 

Now, don't get me wrong. I don't think a positive attitude will prevent scleroderma or even cure it. Rather, that it will just put things in our favor to help our bodies make the best of a bad situation. It drives me bonkers when people over-credit attitude, as though it will make anyone or everyone overcome anything or everything, and thus live forever in perfect health. That is not a positive attitude, that is magical thinking, on a scale that is out of touch with reality.  No, I'm just talking about how attitude can take the edge off things and at minimum make our journey more pleasant for us even if, in the end, it is the same journey that it would be if it was accompanied by a totally negative attitude.

 

Bottom line?  The methotrexate will help you the absolute most if you throw all your belief into it.  It also would be helpful to *expect* that your scleroderma symptoms will relent a bit, just in the natural course of the disease. Take the positive thoughts about methotrexate plus the natural waning of the disease, and you have a LOT to be positive and hopeful about!  And those positive and hopeful thoughts will sway your immune system through all those fancy neuroendocrine or whatever pathways.

 

It's an interesting thing to know what type of scleroderma we have, but it is seldom a necessity, and few of us actually know for absolutely certain what type we have. There is usually a lot of wiggle room between what our symptoms, our blood work, and what our doctor(s) have told us.

 

My conclusion is it has to be extremely common for most of us to be self-identifying with only our best guess. I would also venture to say from my observation of the stories submitted on our main site and our book series, that the majority of people have pretty accurately pegged themselves, when pressed to identify as having either limited or diffuse.

 

Therefore, I say, that if you think you have diffuse scleroderma based on this or that, you are probably right, but it will also probably take years for that to be confirmed by your doctor, and even then, another doctor might disagree with the classification, if, for example, your skin involvement is not yet above your elbows. 

 

So, are you telling the "truth" or not if you tell people you have diffuse scleroderma?  I'd venture to say "yes", because you are basing it on some identifiable fact that means it is more likely than not; with the caveat that it would be true unless your scleroderma expert has put you in another permanent category and explained their reasoning to you.

 

And in this usually fogged-in realm, that's about as good as it can get. I hope it will help you to realize that very few things are carved in stone when it comes to scleroderma types, and that most things about it are grey and not as black and white as most of us would wish.

 

:emoticons-group-hug:


Warm Hugs,

Shelley Ensz
Founder and President
International Scleroderma Network (ISN)
Hotline and Donations: 1-800-564-7099

The most important thing in the world to know about scleroderma is sclero.org.

#6 judyt

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Posted 23 February 2015 - 12:17 PM

Hi All,

 

Well put Shelley, I have to say that when my GP says "this will help" it usually does even if only in my head.   That is if I really think about it, whatever it was that was bothering me is actually still there but I can shut it out because she said so.

 

On the other hand after about 45 years my Raynaud's gradually retreated in 2011.   I began to get fewer attacks, then an attack was just a tingling sensation in my fingers but no colour changes then all of a sudden one day I realised that I hadn't had anything for weeks.

 

I have never had any external skin involvement except for a slight Sclerodactyly, and very very slight contracture of my fingers.    However my internal involvement continues unabated.   I now have confirmed Achalasia as well as all the other things but no Gastroparesis.   Apparently once my food gets to my stomach it moves on fairly quickly, it is getting it to the stomach which is the difficulty.

 

As well I am definitely Anaemic and have had quite a torrid run of low ferritin and haemoglobin necessitating a transfusion of 3 units of red cells and infusions of 2 units of Ferritin since 21 December.   Looks like this might be my routine for the next while.   The plan is for me to have a Balloon Enteroscopy on 17 March to check on internal bleeding in the small bowel and after that a phone call to the hospital whenever my Ferritin gets low and they will book me in for another Infusion.

 

Such is life, at least I am still here and in an earlier generation I probably wouldn't have been so that is something I am inclined to be very thankful for.   I do what I can do and am lucky to have a husband who was able to retire early and who is quite happy to take over in the house and garden - and at the shops - whenever necessary.   Actually I think he might be a shop-a-holic :emoticon-dont-know: because when he had his business he was the one in the office doing all the ordering of parts and organising of labour and he has never given that up :emoticons-clap: .   He manages not to be too bossy but he is good at reminding me about what I should be doing when I can.

 

I wonder if anybody else has had an Enteroscopy??   I always thought that the small bowel was a no go area but I have learned differently just lately.

 

Best wishes to all

From the upside down side of the world

Judyt



#7 Kathy D

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Posted 23 February 2015 - 09:33 PM

Grey,

 

I was told from the beginning it was Diffuse but only had a few shiny patches near my knuckles, though all the other symptoms (all my joints blew up, Raynauds, severe fatigue and unrelenting fever) that got worse over the years).  Now 6 years later the shiny patches are starting to spread, though it's only on my knuckles and part of the top of my hand, and I have odd spots on my face.  My upper lip is distinctive IMHO, and though I can see I am aging quickly in my 40's, my face looks younger than others my age except for the prednisone chin LOL LOL! I have some other weird spots but I assume it's part of the disease.

 

I am over due for Pulmonary Function tests and Echo but a few years ago I was told my Diffuse was behaving as Limited. 


Diffuse Scleroderma Diagnosed March 2009

#8 greypilgrim256

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Posted 24 February 2015 - 07:05 AM

Thanks for the replies.  I put a lot of faith, maybe too much, in antibody testing and statistics.  While I know that they are not the absolute "truth" and there are exceptions, numbers and trends tend not to lie.

 

I definitely feel better than I did this time last year, but the uncertainty of this disease is awful.  The fact remains that men tend to have a worse outcome than women and my antibody type (U3RNP) is extremely rare, tends to have one of the worst mortality rates due to a high incidence of isolated Pulmonary Arterial Hypertension regardless of skin involvement.  

 

With that being said, I do agree with you Shelley about people categorizing themselves as either diffuse or limited.  I also have read from someone that once said "this disease presents itself early" and I'm clinging to that.  

 

Regardless, I am still adopting an approach to be as aggressive with treatment as my doctors will allow. 



#9 Shelley Ensz

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Posted 24 February 2015 - 10:56 AM

Hi Grey,

 

The chief advantage you have, over other people in your same boat, is that your illness has been identified already.  This is extremely significant because you, and your doctors, are already aware of the possible complications you may face, with both scleroderma and pulmonary hypertension. Therefore, you are highly likely to maintain good health habits and appropriate medical care, and identify any complications early on, when they are at their most treatable.

 

This puts everything in your favor for increased longevity under the circumstances. Also, you have sought reliable sources for support, which means you aren't going to be left dangling emotionally, which would make you even more vulnerable to worsened health.

 

Clearly, you have already opted out of the "diagnosed by autopsy" category.  Rather you are in the "living with scleroderma" category and with any luck and a good degree of planning and proper health habits and medical care, I trust you will be one of those who s t r e t c h  those statistics to the maximum.  In this day and age, with improved detection and treatments, that is both possible, and perhaps even likely.

 

:emoticons-group-hug:


Warm Hugs,

Shelley Ensz
Founder and President
International Scleroderma Network (ISN)
Hotline and Donations: 1-800-564-7099

The most important thing in the world to know about scleroderma is sclero.org.

#10 Amanda Thorpe

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Posted 25 February 2015 - 04:10 AM

Hello Shelley

 

I wasn't as it happens, I was referring to the UK definition of diffuse systemic scleroderma because we've only got one and it includes skin involvement over the whole body within months. It's not unusual for a UK/US difference, we differ also regarding the extent of skin involvement needed to differentiate between limited and diffuse.

 

Well I obviously have the worse type of diffuse scleroderma then, according to what constitutes rapid onset diffuse then.

 

Sorry if I caused and confusion.

 

Take care,


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#11 lizzie

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Posted 25 February 2015 - 07:12 AM

I was initially diagnosed with limited Scleroderma (in the UK) as I only had thickened skin on my fingers and face (also had positive ANA - nucloelar pattern).  About 18 months later my skin began to rapidly tighten on my arms and lower legs and my rheumatologist decided that I needed to start immunosupression ( I had Cellcept , then Methotrexate) which really worked well for me. The rheumatologist told me that I had something between limited and diffuse scleroderma! I've hardly any thick /tight skin now - mainly on my face which at my age is a bonus as keeps the wrinkles at bay!



#12 Joelf

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Posted 25 February 2015 - 09:12 AM

Hi Lizzie,

Long time.....no hear! ;) How lovely to hear from you again and I do hope that you're doing okay. :)

I know what you mean by wrinkles; the Prednisolone I take causes my face to fill out, so that does push out a few face creases. There's always a bonus if you look for it!!

Although my lungs are affected, I have minimal skin involvement and my rheumatologist diagnosed Limited Scleroderma. It does seem to have been fairly constant for the last five years.

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#13 lizzie

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Posted 25 February 2015 - 01:27 PM

Hi Joelf, I've been really well over the last couple of years-even the Raynauds seems to be much better! Still working full-time but currently on sick leave as sustained a trimalleolar ankle fracture slipping on wet grass when shutting the hens away.

#14 Shelley Ensz

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Posted 25 February 2015 - 07:00 PM

Lizzie, hello it's great to hear from you again!

 

Amanda, yes, it is the different systems for diagnosis used hither and yon, and also changed over time, that makes the categories for limited and diffuse more of a moving target.  You have what would likely be called rapid onset diffuse scleroderma in the U.S.

 

So people should be aware that their officially designated type of scleroderma may sometimes change only because they change doctors or move to other countries, even if their illness hasn't changed in any way.  And Lizzie's case illustrates the gray area, which basically means scleroderma is sometimes going to do whatever it pleases, regardless of how it is labeled (or not) at the moment.

 

Unfortunately, our bodies more often than not fail to read the textbook description for scleroderma, and creatively do their own thing, either worsening or healing on their own accord.

 

:emoticons-group-hug:


Warm Hugs,

Shelley Ensz
Founder and President
International Scleroderma Network (ISN)
Hotline and Donations: 1-800-564-7099

The most important thing in the world to know about scleroderma is sclero.org.

#15 quiltfairy

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Posted 25 February 2015 - 09:21 PM

Hi

 

My scleroderma has also been slow, all I can say about is that I am thankful for that.   I do have swelling in my hands, my joints ache most of the time, but I am thankful because I can still do as I wish for my sewing projects.

 

I also have diffuse, any way that is what my doc said.

 

I used to think that this was a death sentence, I worried about how the disease was progressing and if it did not follow the book my doc, was getting a call.  I have calmed down now, this is not a death sentence but it is a little uncomfortable.

 

Only problem with it being slow is that my brother calls me an hypochondriac because he hears about the illness but he cannot see it.   My hope above all else is that  your scleroderma and everyone elses slows down to a crawl. 



#16 greypilgrim256

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Posted 26 February 2015 - 06:20 AM

Thanks for the input everyone. Lizzie, do you happen to know what antibody type you have with the nucleolar pattern?  Have you ever had "tendon friction rubs" at any of your joints?  



#17 Shelley Ensz

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Posted 26 February 2015 - 09:02 AM

Quiltfairy, if everyone here with scleroderma who has been called a hypochondriac by their friends or family raised their hand and we took a screen shot of it, there probably wouldn't be a computer screen anywhere wide enough to display the whole picture.

 

Although hypochondria is actually quite rare, inaccurate armchair diagnosis by unqualified people is probably at epidemic proportions, and it is typically helped along by the truly drastic delay in accurate diagnosis for scleroderma.

 

But, it is what it is. You can't fight a "diagnosis" of hypochondria by explaining more about your symptoms, because that just affirms the diagnosis in their mind. What I  do is try to silently forgive them for being terminally stupid, and in the case of relatives, I really hope that the terminal stupidity is not genetic.

 

And if anyone has found a more effective way to deal with this all-too-common situation, we'd surely all love to hear about it.

 

:emoticons-group-hug:


Warm Hugs,

Shelley Ensz
Founder and President
International Scleroderma Network (ISN)
Hotline and Donations: 1-800-564-7099

The most important thing in the world to know about scleroderma is sclero.org.

#18 judyt

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Posted 26 February 2015 - 10:22 AM

Hi,

 

I explained once to a moderately sympathetic relly, wife of a cousin, how I feel.   

 

They live the life of Riley, at sea most of the time or rather holed up in some fabulous cove somewhere where it is hot, but not too hot, living on board their ocean going yacht!!  In spite of the thought of that luxury they have to be really fit and able and ready for any eventuality.  

 

So I said to her, do you ever get so exhausted that you feel you can't go on, can't take another step before you have rested??   Of course she has.   Well that's how I feel 90% of the time.

 

Nuff sed

Judyt



#19 quiltfairy

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Posted 26 February 2015 - 01:52 PM

I simply told my brother I would change places with him any day.   I would love to be putting in my 40 hours a week and he could sit home on disability trying his best to cope with a disease like I have.

 

 

He did not seem to want to do that.



#20 lizzie

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Posted 27 February 2015 - 06:59 AM

Hi greypilgrim, in response to your question- although I have nucleolar pattern ANA the tests have not detected any specific antigen. However the standard testing in my hospital only tests for some but not all antigens.