00:00
Our topic here is scleroderma. If it's one description that was to be given to scleroderma,
it would be fibrosis. Let's take a look. Definition. It's exactly what it is, why it is fibrosis,
but what if it was limited to the skin, then there'd be something called limited type of
scleroderma and they'd be part of a constellation called CREST syndrome and if it was,
remember CREST, C stood for calcinosis and you can also use that C for the marker for limited
type of scleroderma which is anticentromere, the R would be for Raynaud, E esophageal
dysmotility, S sclerodactyly, and T telangiectasia. So we're about to talk about scleroderma.
00:45
Let's begin. Sclero means scarring, derma of course we are referring to the skin; however,
it doesn't always have to be limited to the skin, does it. So, if you have organ involvement or
visceral type of sclerosis, then no doubt we have systemic. Unknown in terms of etiology,
scleroderma usually affects middle-aged women autoimmune. Early lesions demonstrate
vascular endothelial damage associated with autoantibodies and we'll talk about what those
are in a second. Steroids will not be effective here because you have unlimited production of
fibrosis. There are 2 types now, the systemic, the diffuse type. The diffuse type by definition
not only would you have skin involvement but then also organs. The marker that we'll talk
about with diffuse is called Scl-70. You may want to think about it as scleroderma 70. However,
another name for Scl-70 is topoisomerase I. The limited type, what does that mean? You would
have fibrosis taking place exclusively to the skin or only to the skin, maybe in the forearm.
01:56
Also has early development of pulmonary hypertension, there is a possibility, and you have
small-vessel damage. So, the biggest difference, make sure that you know diffuse type which
you have no doubt rampant and technical organ involvement, much more aggressive. That's the one
that may kill your patient. If it's a limited type, skin, maybe the blood vessels. In the limited
type, we have CREST. It's what C-R-E-S-T stands for. The symptoms are characterized by the
following, let's talk about that CREST and let's first talk about Raynaud. Now Raynaud disease
would mean if it's primary. The Raynaud disease will be primary which means that there was no
underlying issue, it's not secondary. It will just be idiopathic. If it's Raynaud phenomenon,
then it would be part of limited. Look for calcinosis, esophageal dysmotility, "doc, I'm having a
hard time eating, hmmm." Take a look at the fingers and I'll show you in a little bit and it looks
like the fingers of that of a witch, tapered, sclerodactyly. Now what happens first is the fact
that you take the hand and you place it into the cold. Upon exposure to the cold, the little
blood vessels in your fingers will start undergoing vasospasms, hmm. So now if the blood
vessels within your digits undergo vasospasms, you're decreasing the supply of oxygen to
your digits, correct. What's the 1st color do you think it'll first become? That's important. It
won't be cyanotic immediately, will it, no, but it'll begin the process of cyanosis in the fingers.
03:30
So the 1st color you're going to go from, take a look at your finger here now, it's pinkish,
right. You should have capillary filling and such. You put it out in the cold, at first vasospasms
might then cause it to be white. Continued exposure and vasospasm will then cause it to
become blue which is your cyanosis. You bring the hand back into the warmth, you're returning
blood supply to the fingers. Now, it goes from being blue to dusky, ah, to red. That's the
spectrum of colors that you want to know for Raynaud, white, blue, red, the colors of the flag;
however, the order is a little bit different, right. Raynaud phenomenon, vasospasms taking
place in your digits upon exposure to the cold. Sclerodactyly in CREST, the S, it means that
the finger is undergoing tapering, thickening that I'll show you in a little bit. It's called
sclerodactyly. Decreased facial skin, wrinkles because of, once again, scleroderma. Esophageal
dysmotility, back to your overgrowth malabsorption syndrome. That's a possibility as well
with CREST. You must understand that with CREST the type of scleroderma that you call this is
limited in which the marker for this is ____ . Here we have the diffuse type of scleroderma.
05:03
Hmmm, things become a lot worse here. Not only may you have involvement of the skin but
then you have end-organ involvement. Hmmm, imagine if the lung became fibrosed and you
have decreased compliance. It'd be a very hard cased lung. In other words, you'll just form
interstitial lung disease in which the alveoli may then perish, eventually may lead in to
pulmonary hypertension. Oh boy, if that happens you'll be worried about right-sided heart
failure, hemodynamic instability, not a good situation for the patient to be in. It could cause
death. It could be cardiomyopathy of what kind please, restrictive. You just fibrosed the heart
for Pete's sake. Next, there might be a fibrosis of intestine. That definitely is going to cause
malabsorption and what you're worried about is your kidney. Could you imagine if fibrosis is
taking place down in the renal? You lost your GFR, hypertension, may then go into RPGN within
3 months, 12 weeks, those kidneys are gone. Not good. You take a look at the fingers here,
limited, and with limited, we have the arrows that are pointing to the areas that are fibrosed,
tapered, thickening taking place. This is known as sclerodactyly. The type of inflammation
that I showed you earlier on the hands, let's say of rheumatoid arthritis or even psoriatic
arthritis, that was called dactylitis. That was inflammation. This is smooth, tapered fingers of
the hand. Let's take a look at that nail fold even closer. So imagine now that the top portion
of the 1st picture on the left represented the nail then down here is the cuticle and then
within your cuticle obviously will then be your capillaries. The 1st picture on your left is normal.
07:03
Now, with sclerodactyly, you'll notice now that there's capillary dilation taking place in the
middle picture and then there's something called capillary dropout. It is a part of the changes
that are taking place with sclerodactyly. Diagnosis is scleroderma. Characteristic examination,
look for the skin changes that we talked about where there's taut skin because of fibrosis.
07:29
ANA is usually positive but keep in mind that obviously it's not specific. Anti-Scl-70 is going to
be specific for what type of scleroderma, diffuse. Another name for Scl-70 is anti-topoisomerase I.
07:47
If you find the marker being anticentromere antibody then this will be the limited type of
scleroderma which will then be present in CREST. Management, really no effective treatment
unfortunately available. Calcium channel blockers, we'll then be thinking of being used with
Raynaud if the patient is suffering from limited type of scleroderma and Raynaud. Esophageal
reflux, with the reflux, then you're thinking about perhaps using PPI, and if you're worried
about renal issues, then you should be thinking about ACE inhibitors. Steroids and
cyclophosphamide for interstitial lung disease, you're thinking about your modulators. The skin
stretching exercises because things are becoming, even though the patient may look young
it's pathological, so you need to make sure that you maintain proper mobility, and endothelin
receptor antagonist, bosentan. Remember endothelin is an endogenous component that is
being formed by the endothelial cell responsible and an extremely potent vasoconstrictor.
08:57
An endothelin-receptor I blocker such as bosentan could be used for the management of
pulmonary artery hypertension that may take place maybe secondary to diffuse scleroderma.