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 you can find written notes on this topic at

zero to finals.com slash systemic sclerosis or in the rheumatology section of the second edition of the

zero to finals medicine book. So let's get straight into it.

Systemic sclerosis is an autoimmune connective tissue disease involving inflammation and fibrosis,

which is hardening or scarring, of the connective tissues, the skin and the

internal organs. The cause is unclear. The term scleroderma translates directly to

hardening of the skin. The terms systemic sclerosis and scleroderma are often used interchangeably.

Most patients with scleroderma have systemic sclerosis. However, there is a localized version of

scleroderma that only affects the skin. There are two main disease patterns in systemic sclerosis, limited cutaneous systemic sclerosis and

diffuse cutaneous systemic sclerosis. So let's start with limited cutaneous systemic sclerosis.

Limited cutaneous systemic sclerosis is the more limited version of the disease.

It used to be called Crest syndrome, and Crest forms a mnemonic for remembering the features of limited cutaneous systemic sclerosis.

C for calcinosis, R for Raynard's, E for esophageal dismotility, S for scleridactyl, and T for

telangetacea, and we'll talk in more detail about all of these features shortly.

Next let's discuss diffuse cutaneous systemic sclerosis.

Diffuse cutaneous systemic sclerosis includes the crest features, but it also affects

the internal organs, causing cardiovascular problems, for example, hypertension and

coronary artery disease, lung problems, for example, pulmonary

hypertension and pulmonary fibrosis, and kidney problems, for example, glomerulinephritis,

and scleroderma renal crisis. So let's talk in more detail about the features.

Scleroderma refers to hardening of the skin, giving the appearance of shiny, tight skin without

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