Title: Limited Scleroderma (CREST Syndrome)
Headings: Limited Scleroderma (CREST Syndrome)
Limited Scleroderma (CREST Syndrome)
Overview
Causes and Risk Factors
Symptoms
Diagnosis and Treatment
Prevention
Sources
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Content: Limited Scleroderma (CREST Syndrome)
Limited Scleroderma (CREST Syndrome)
By: Tina Shahian, PhD
Last Updated: Mar 1, 2019
Overview
Limited scleroderma (also known as CREST syndrome) is a type of scleroderma, which as the name implies is characterized by thick, hardened skin. In the more severe form, this connective tissue disorder can affect the function of internal organs (e.g., esophagus, heart, lungs, gastrointestinal tract, muscle and joints) and even become life threatening. Limited scleroderma is milder form of the disease and affects roughly 50% of scleroderma patients. It is often referred to as CREST syndrome, with each letter representing one of five common features ( see Symptoms) of the illness: C alcinosis
R aynaud phenomenon (also called Raynaud’s phenomenon or Raynaud’s disease)
E sophageal dysfunction
S clerodactyly
T elangiectasia
The skin hardening associated with limited scleroderma is typically confined to the fingers, hands, lower arms, legs and face. Unlike its more severe form, progression is more gradual and complications involving internal organs are less common. Limited scleroderma can affect the digestive tract, causing difficulty swallowing and frequent heartburn. It can also affect the blood vessels, heart and lungs, leading to hypertension and shortness of breath.