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Limited or diffuse?


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#1 dimarzio

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Posted 09 February 2016 - 08:21 AM

I'm a little unsure of when limited becomes diffuse.  Is it still limited if affects the esophagus.  Is it still limited if it affects the stomach and the colon?

 

I know that diffuse can affect the lungs, heart and kidneys but was unsure (from what I've read) whether gastro problems fell in to the limited or diffuse category.



#2 Shelley Ensz

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Posted 09 February 2016 - 10:36 AM

Hi Dimarzio,

 

It's up to your scleroderma expert to figure out whether you have limited or diffuse, but it seems to be very common for people with systemic sclerosis to not know exactly which subtype they have. If I recall correctly, a survey in the UK showed that about half of systemic patients had not been told whether they had limited or diffuse.

 

The thing is, both limited and diffuse can affect any of the internal organs. So the primary difference in categorizing is that limited means that the skin involvement is more limited, in that it is confined to the hands, feet and/or face.  Whereas diffuse can cause skin tightening even above the elbows and knees and on the trunk, etc.

 

The worst form of scleroderma is a very rapidly progressing diffuse scleroderma. Within weeks, it can cause widespread skin tightening. Generally speaking, they figure the skin involvement can sometimes be reflective of the damage to internal organs. But there are exceptions to every rule, and some people can have primarily skin tightening, and others can have a lot of internal organ involvement but little or no skin involvement.

 

Basically, if you are still alive a few months after onset, and your skin isn't tightened from head to toe, then you do not have the absolute worst kind of scleroderma. It is entirely "normal" for systemic scleroderma, of either type, to affect the entire gastrointestinal tract, from stem to stern.

 

It's also normal for things to be worse during the onset stage, like the first year or two. I definitely know how it's rather alarming to get new symptoms every whipstitch!  But then things often settle down, even on their own accord, and usually resemble more of a chronic illness than a rapidly progressive, downward spiral illness.  Most of us will sprout our most prominent symptoms in the first few years, and then be left to deal with the fallout on an ongoing basis.

 

For more information on this topic, we have lots of educational videos, especially about the Types of Scleroderma.  But no matter what I say about what type and what classification, it's up to your scleroderma expert to define what type you have. Don't be surprised if they sit on the fence about that for a few years, waiting to see how things develop.

 

On the positive side, the normal course of scleroderma is for it to wax and wane. That means, when we are in a spell of worsening, we can be hopeful because, with proper care (which can even include rest and relaxation), it is likely that we will have a spell of improvement, right around the next bend! 

 

:hug-group:


Warm Hugs,

Shelley Ensz
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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.

#3 dimarzio

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Posted 10 February 2016 - 05:03 AM

That's very helpful, Shelley and perhaps gives me a little hope.  The weather seems to have a huge impact and when it's mild enough to get out for a walk, I feel a less bad.  But a few days of wind and rain and my head is spinning from the pain of stiffening joints.  When it's windy, I can feel draughts in the house that nobody else seems to feel, but with all the doors and windows shut, the internal doors are banging, I just can't seem to locate where the draught is coming from.

 

I don't have a huge amount of visible skin involvement save for one of my feet and a small patch on my hand. I'm starting to feel some skin changes between my shoulder and upper chest which is a little concerning - nobody else can feel it, but I can feel that it's different to the rest of my skin and how it was 6 months ago.

 

I'm also a bit concerned that doctors are fobbing off my constant bloatedness and constipation as IBS when I've never had IBS before getting this illness.  They are investigating my esophagus further though.



#4 Choclit

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Posted 26 February 2016 - 03:29 AM

It is my understanding that while this is not completely consistent among scleroderma researchers, the new trend is to use antibodies as the primary determinant of scleroderma subtype.  Historically, only two antibodies where know to be associated with scleroderma: centromere and Scl-70.  Patients with centromere antibodies typically have later skin involvement and it is almost always "limited" to hands (sometimes up to the elbows), feet, and face.  In contrast, patients with Scl-70 antibodies have skin changes that were more "diffuse", including the trunk.  This is where the names Limited Cutaneous Systemic Scleroderma (lcSSc) and Diffuse Cutaneous Systemic Scleroderma (dcSSc) comes from.  When the RNA Polymerase III antibody was identified, it also has diffuse skin changes.  Later Th/To antibodies were identified as another rare scleroderma related antibody and the skin pattern changes were similar to centromere.

 

Now there are about eight different scleroderma related antibodies plus about 6% of patients with clear scleroderma symptoms but test ANA negative even by the IFA method.  They are divided into three "camps": diffuse, limited, and overlap syndromes (such as Mixed Connective Tissue Disorder).  Antibody type doesn't change and it is very rare (less than 2%) for a patient to actually have more than one scleroderma related antibody.

 

Some clinicians still look at the skin changes as diagnostic, which leads to a great deal of confusion where patients are told that they are limited but later changed to diffuse as skin changes progress.  I am in complete agreement with the modern trend of using antibodies as the primary classification method.

 

Here is a link to an antibody table within my Scleroderma FAQ that lists Scleroderma related antibodies and how they are classified, as well as some significant information about risks with each type.


Ed Harris
Scleroderma Education Project
A member of the ISN's Scleroderma Webmasters Association (SWA)
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#5 Tully

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Posted 04 March 2016 - 09:20 PM

Hey dimarzio,

 

When I was officially diagnosed with diffuse SD, the doc pinched my skin in 17 spots over my body. A score of "0" for normal and a "3' for tight. I scored 24, anything over 17 he considered diffuse. That was Sept 08, when I had my bone marrow transplant Dec '10 I scored a 46. My current doc doesn't keep score but I would estimate I'm down in the low 30s now.