Hello and welcome to the zero to finals podcast.

My name is Tom and in this episode I'm going to be talking to you about systemic sclerosis.

And if you want to follow along with written notes on this topic,

you can follow along at zero to finals.com slash systemic sclerosis

or in the rheumatology section of the zero-definals medicine book.

So let's get straight into it.

The terms systemic sclerosis and scleroderma are often used interchangeably.

Most patients who have scleroderma have systemic sclerosis.

However, there is a localized version of scleroderma that only affects the

skin and is not technically systemic sclerosis. Scleroma translates directly to hardening of the skin.

Systemic sclerosis is an autoimmune inflammatory and fibrotic connective tissue disease. The cause of the condition is unclear. It most

notably affects the skin in all areas, but it also affects the internal organs. There's two main

patterns of disease in systemic sclerosis, limited cutaneous systemic sclerosis and diffuse cutaneous systemic sclerosis.

First, let's talk about limited cutaneous systemic sclerosis.

And this is the more limited version of systemic sclerosis.

It used to be called crest syndrome,

and this forms a helpful mnemonic for remembering the features.

The mnemonic is Cres C is for calcinosis and this is calcium deposits under the skin.

R is for Raynard's phenomenon. E is for esophageal dismotility.

S is for sclerodactyl and T is for telangjectasia and we'll go through what all of these mean just shortly.

Next is diffuse cutaneous systemic sclerosis and this includes all the features of Crest syndrome plus it affects the internal organs causing cardiovascular problems, particularly hypertension and coronary artery disease,

lung problems, particularly pulmonary hypertension and pulmonary fibrosis, as well as kidney problems,

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