Scleroderma: Limited vs. Diffuse

by Carlo Raj, MD

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    00:01 Our topic here is scleroderma.

    00:03 If it’s one description that was to be given to scleroderma, it would be fibrosis.

    00:09 Let’s take a look, definition.

    00:12 That’s exactly what it is, it’s widespread fibrosis.

    00:15 But what if it was limited to the skin? Then that’d be something called limited type of scleroderma.

    00:21 And then it would be part of a constellation called CREST syndrome.

    00:25 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 Anti-centromere.

    00:36 The R would be for Raynaud, E, Esophageal Dysmotility, S, Sclerodactyly, and T, Telangiectasia.

    00:43 So we have a lot to talk about scleroderma, let’s begin.

    00:46 Sclero means scarring, derma of course, we’re referring to the skin.

    00:51 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.

    01:02 Unknown in terms of etiology.

    01:05 Scleroderma usually affects middle-aged women, autoimmune.

    01:08 Early lesions demonstrate vascular endothelial damage.

    01:12 Associated with autoantibodies and we’ll talk about what those are in a second.

    01:17 Steroids will not be effective here because you have unlimited production of fibrosis.

    01:22 There are two types now.

    01:25 The systemic and the diffused type.

    01:28 The diffused type by definition, not only would you have skin involvement but then also organs.

    01:33 And markedly we’ll talk about what diffused is called SCL70.

    01:37 You may wanna think of it as scleroderma 70.

    01:40 However, another name for SCL70 is topoisomerase 1.

    01:44 The limited type, what does that mean? You have fibrosis taking place exclusively to the skin or only to the skin, maybe in the forearm.

    01:55 Also has early development of pulmonary hypertension.

    01:59 There is a possibility.

    02:01 And you have small vessel damage.

    02:05 So the biggest difference, make sure that you know diffuse type which you have no doubt rampant and technical organ involvement, much more aggressive.

    02:16 That’s one that may kill your patient.

    02:18 If it’s a limited type skin, maybe the blood vessels.

    02:22 In the limited type, we have CREST is what C-R-E-S-T stands for.

    02:27 The symptoms are characterized by the following.

    02:30 Let’s talking about that CREST and let’s first talk about Raynaud.

    02:34 Now, Raynaud disease would mean if it’s primary.

    02:38 So Raynaud disease would be primary which means that there was no underlying issues on secondary.

    02:43 It would just be idiopathic.

    02:45 If it was Raynaud phenomenon, then it will be part of limited.

    02:49 Look for calcinosis, esophageal dysmotility, “Doc, I’m having a hard time eating.” 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.

    03:01 Now, what happens first is the fact that you take the hand and you place it into the cold.

    03:05 Upon exposure to the cold, the little blood vessels in your fingers will start undergoing vasospasms.

    03:10 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 first color do you think it will first become? That’s important.

    03:23 It won’t be synodic immediately, will it? No, but to begin the process synosis in the fingers.

    03:29 So the first color, you’re going to go from - take a look at your finger here now.

    03:34 It’s pinkish, right? You should have capillary filling and such.

    03:37 You put it out into the cold at first, vasospasms might then cause it to be white, continued exposure and vasospasms will then cause it to become blue which is your cyanosis.

    03:49 You bring the hand back into the warmth, you’re returning blood supply to the fingers.

    03:55 Now, it goes from being blue to dusky, to red.

    04:01 That’s the spectrum of colors that you wanna know for Raynaud, white, blue, red.

    04:08 The colors of the flag, however, the order’s a little bit different, right? Raynaud phenomenon, vasospasm’s taking place in your digits upon exposure to the cold.

    04:24 Sclerodactyly, in CREST, the S.

    04:28 It means that the fingers are undergoing tapering, thickening that I’ll show you in a little bit.

    04:35 It’s called sclerodactyly.

    04:36 Decreased facial skin, wrinkles, because of once again, scleroderma.

    04:43 Esophageal dysmotility, bacterial overgrowth, malabsorption syndrome, that’s a possibility as well with the CREST.

    04:51 You must understand that with the CREST, the type of scleroderma that you call this is limited in which the marker for this is sent.

    05:00 Here we have the diffused type of scleroderma.

    05:03 Things have become a lot worse here.

    05:06 Not only may you have involvement of the skin, but then you have end-organ involvement.

    05:11 Imagine if the lung became fibrosed, you have decreased compliance.

    05:16 You have a very hard cased lung.

    05:18 In other words, you just formed interstitial lung disease in which the alveoli may then perish, eventually may lead into pulmonary hypertension, oh boy, and if that happens, you’re worried about right sided heart failure, hemodynamic instability, not a good situation for the patient to be in.

    05:34 It could cause death, it could be cardiomyopathy.

    05:38 Of what kind please? Restrictive.

    05:40 You just fibrosed a heart for Pete’s sake.

    05:42 Next, there might be fibrosis of intestine.

    05:46 That definitely is gonna cause malabsorption and what you’re worried about is your kidney.

    05:53 Would you imagine if fibrosis is taking place down in the renal? You’ve lost your... due for hypertension, you may then go into RPGN within three months, 12 weeks, those kidneys are gone, not good.

    06:08 If you take a look at the fingers here, limited.

    06:13 And with limited we have the areas that are pointing to the areas that are fibrosed, tapered, thickening taking place also known as sclerodactyly.

    06:26 The type of inflammation that I showed you earlier in the hands, let’s say rheumatoid arthritis or even thoracic arthritis.

    06:34 That was called dactylitis.

    06:37 That was inflammation.

    06:38 This is smooth tapered fingers of the hand.

    06:43 Let’s take a look at that nail fold even closer.

    06:46 So imagine now that the top portion of the first picture on the left represented the nail and then down here is a cuticle, and then within your cuticle obviously would then be your capillaries.

    07:00 The first picture on your left is normal.

    07:02 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.

    07:12 It is all part of the changes that are taking place with sclerodactyly.

    07:16 Diagnosis of scleroderma, characteristic examination.

    07:20 Look for the skin changes that we talked about where there’s taut skin because of fibrosis.

    07:28 ANA is usually positive but keep in mind that obviously, it’s not specific.

    07:33 Anti-Scl-70 is going to be specific for what type of scleroderma? Diffused, another name for Scl-70 is anti-topoisomerase-1.

    07:46 If you find a marker being anti-centomere antibody, then, this will be the limited type of scleroderma which will then be present in CREST.

    07:55 Management really no effective treatment unfortunately available.

    08:00 Calcium channel blockers would then be thinking of being used with Raynaud if the patient’s suffering from limited type of scleroderma in Raynaud.

    08:10 Esophageal reflux with that 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.

    08:24 Steroids and cyclophosphamide for interstitial lung disease, you’re thinking about your modulators.

    08:31 The skin stretching exercises, those things are becoming - even though the patient may look young, it’s pathological.

    08:38 You need to make sure that you maintain proper mobility and endothelin-1 receptor antagonist, bosentan.

    08:46 Remember, endothelin is an endogenous component that is being formed by the endothelial cell responsible and an extremely potent vasoconstrictor.

    08:57 An endothelin-1 receptor blocker such as bosentan could be used for the management of pulmonary arterial hypertension that may take place maybe secondary to diffused scleroderma.

    About the Lecture

    The lecture Scleroderma: Limited vs. Diffuse by Carlo Raj, MD is from the course Connective Tissue Diseases. It contains the following chapters:

    • Introduction to Scleroderma
    • Limited Scleroderma
    • Diffuse Scleroderma

    Included Quiz Questions

    1. CREST syndrome
    2. Rheumatoid arthritis
    3. Mixed connective tissue disorder
    4. Systemic Lupus Erythematosus
    5. Diffuse type of scleroderma
    1. Calcification of long bones
    2. Dysphagia
    3. Sclerodactyly
    4. Raynaud’s phenomenon
    5. Calcinosis
    1. Anti-Scl70
    2. Anti-centromere
    3. Anti-histone
    4. ANA
    5. RNP.
    1. Hydrocortisone
    2. Nifedipine
    3. Amlodipine
    4. Isradipine
    5. Felodipine
    1. Prednisone
    2. Nifedipine
    3. Omeprazole
    4. Bosentan
    5. Epoprostenol

    Author of lecture Scleroderma: Limited vs. Diffuse

     Carlo Raj, MD

    Carlo Raj, MD

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