Scleroderma

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.