by Carlo Raj, MD

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    00:01 Scleroderma is the next topic. Scleroderma in general, here because you have the viscera being involved.

    00:10 That 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. The limited would be one in which you would then find skin involvement.

    00:25 The limited type would be skin without any viscera involvement. The skin type of scleroderma is involved with CREST. That's a different topic with calcinosis, Raynaud's, esophageal dysmotility, sclerodactylyl, and telangiectasia. Here with scleroderma if you're thinking about viscera involvement, not only should you be thinking about the esophagus, more importantly you're thinking about the diaphragm and also the kidney. Cause here, the patient may actually die of renal failure or maybe perhaps respiratory failure. But understand here that there will be diffuse fibrosis leads to loss of lower esophageal sphincter function. There is complete absence of peristalsis, esophagel dysmotility.

    01:11 The symptoms of GERD and dysphagia are very much present. 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. That should not be the case and it is hard as a pipe. Therapy PPI, anti-reflux measure. Obviously here you're thinking about an autoimmune disease. And with scleroderma here if it's a difuse and visceral 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: Basic Principles with Carlo Raj.

    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. A normal resting pressure of the lower esophageal sphincter
    1. Scl-70 and anti-topoisomerase
    2. ESR and C-reactive protein
    3. Antinuclear antibody
    4. Anti-SS-B
    5. CD19 and CD20
    1. Omeprazole and smoking cessation
    2. Cimetidine and omeprazole
    3. Nonsteroidal anti-inflammatory drugs
    4. Omeprazole only
    5. Antacids

    Author of lecture Scleroderma

     Carlo Raj, MD

    Carlo Raj, MD

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    Presnted very well
    By Dvsvb R. on 04. January 2019 for Scleroderma

    A rare topic presented very well. It is very accurate to understend for any one