by Carlo Raj, MD

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    00:01 Scleroderma is the next topic.

    00:04 Scleroderma in general.

    00:06 Here, because you have the viscera being involved, then we have to refer to what’s known as diffuse or systemic type of scleroderma versus your limited.

    00:18 The limited type would be cutaneous.

    00:21 Limited would be one in which you would then find skin involvement.

    00:25 Limited type would be skin without any viscera involvement.

    00:30 The skin type of scleroderma is involved with CREST.

    00:32 That’s a different topic, with Calcinosis, Raynaud’s, Esophageal dysmotility, Sclerodactyly and Telangiectasia.

    00:40 Here with scleroderma, if you’re thinking about visceral involvement, not only should you be thinking about the esophagus, more importantly you’re thinking about the diaphragm, and also the kidney, because here the patient may actually die of renal failure or maybe perhaps respiratory failure.

    01:00 But understand here that there will be diffused fibrosis, leads to loss of lower esophageal sphincter function.

    01:07 There’s complete absence of peristalsis; Esophageal dysmotility.

    01:11 The symptoms of GERD and dysphagia are very much present.

    01:15 It is associated with Raynaud’s.

    01:18 Manometry shows incompetent lower esophageal sphincter, absent peristalsis because of well, the esophagus has now become a lead pipe.

    01:28 That should not be the case.

    01:29 And it is hard as a pipe.

    01:33 Therapy - PPI, anti-reflux measure.

    01:37 Obviously here, you’re thinking about an autoimmune disease and with scleroderma… here if it’s diffuse and viscera involvement, if you remember correctly, here the marker that you’re going to be looking for is Scl-70 - Scleroderma-70, and topoisomerase.

    About the Lecture

    The lecture Scleroderma by Carlo Raj, MD is from the course Esophageal Disease.

    Included Quiz Questions

    1. Incompetent lower esophageal sphincter and absent peristalsis.
    2. Increased lower esophageal sphincter pressure.
    3. Diffuse spasm of the esophagus.
    4. Decreased lower esophageal sphincter pressure only.
    5. Resting pressure of the LES is normal.
    1. Scl-70 and topoisomerase
    2. ESR and C-reactive protein
    3. Anti-nuclear antibody
    4. Anti-SS-B
    5. CD19 and CD20
    1. Omeprazole and smoking cessation.
    2. Cimetidine and Omeprazole.
    3. Non-steroid anti-inflammatory drugs.
    4. Omeprazole only.
    5. Antacids.

    Author of lecture Scleroderma

     Carlo Raj, MD

    Carlo Raj, MD

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