So Many Different Types of Scleroderma - Mine Is Called Systemic Sclerosis

So I bought this book by the top Rheumalogist/Researcher of Scleroderma here in Houston and there is so much to learn.  She does an amazing job of explaining the 2 main types of Scleroderma which can each be broken down into 3 sub categories.....so technically there are about 6 different variations of the disease which confused me even more, LOL!

The variation I have is called Systemic Sclerosis or Systemic Scleroderma which as explained earlier affects internal organs as well as a small portion of the skin (hands and feet).  My questions were.....what exactly does the collagen do and why does it "overproduce"?  And why does the disease effect the heart, lungs, kidney and GI tract?  Unfortunately, the why part of my question doesn't have an answer which is why I don't have my magic pill.  In order to understand why these things are effected it helped to first understand what collagen is; Collagen is a natural protein that is made by cells and deposited outside of the cell.  It is what makes your skin firm, and it is the major component of connective tissue.  Connective tissue is skin, tendons, joints, ligaments and "capsules" around organs - it is what holds you together.  When you get a cut, collagen is laid down to form the scar.

As explained by Maureen Mayes, "The problem in Scleroderma is that cells start making collagen as if there is an injury that needs to be repaired, even though no such injury has occurred.  And once started, the cells don't turn off.  In systemic scleroderma this happens first in the skin, and then can occur in the lungs, the gastrointestinal tract, the muscles, and elsewhere in the body.  This excess collagen gets in the way of normal functioning.  The fingers don't bend well because of the collagen buildup in the skin; the lungs can't exchange oxygen for carbon dioxide effectively because there is a thick layer of collagen where a very thin membrane is supposed to be; the esophagus and bowels can't move effectively because there is scar tissue where muscle fibers are supposed to be and scar tissue in the heart can interfere with the usual electrical conduction system and cause an irregular or slow heartbeat."

She also goes on to explain that those with Systemic Scleroderma have the extra whammy of having to deal with vascular disease which causes the Raynaud's phenomenon and kidney disease.  Basically, the small blood vessels narrow and sometimes the blood channel is totally closed.

I hope this is helpful.  When people ask why, what and how it is hard to explain.

Next week I have a follow up appointment to see if my lungs have improved since treatment and I will also meet a new dermatologist that uses a special UV therapy on skin to break down collagen.     Hopefully, I will get to use my pinky fingers again, LOL!  I have an Echocardiogram scheduled for April but I may go ahead and move that up so my Rhuemy can give me the results in April.  I will update everyone then.......unless I get more words of wisdom from Maureen Mayes.