Understanding CREST Syndrome

By Semko, Laura 
November 18, 2017

Understanding CREST Syndrome 

CREST syndrome is a type of scleroderma. It’s sometimes called limited cutaneous scleroderma. 

Scleroderma refers to a number of conditions that cause an abnormal buildup of collagen in the body. Too much of this protein makes your skin and other connective tissues thicken and harden. In some cases, it can also affect your organs, such as the lungs and heart. 

CREST syndrome is an autoimmune disease. That means the immune system is not working the right way. It attacks itself. This rare health problem is more likely to happen in women. It often strikes people in their 30s to 50s. 

What causes CREST syndrome? 

Doctors don’t know the exact cause of CREST syndrome. It may be caused by genes or hormones. Some research suggests that a virus may trigger it. People exposed to radiation and certain chemicals such as pesticides or solvents have a higher risk for it. 

Symptoms of CREST syndrome 

CREST stands for its group of symptoms. These are calcinosis, Raynaud syndrome, esophageal dysmotility, sclerodactyly, and telangiectasia. 

These symptoms tend to develop over time. You may first notice changes to your skin on your hands, legs, and face. You may also have problems swallowing. Symptoms may include:

  • Calcinosis. Small bumps may form under your skin. They are made of calcium.

  • Raynaud syndrome. You may lose blood flow to your hands and feet when you are cold or under stress.  This may cause your hands and feet to turn white and then blue. Color returns when they warm back up. You may feel some pain when this happens.

  • Esophageal dysmotility. The extra collagen in your body may break down the muscles in your esophagus, the tube that links your mouth to your stomach. As a result, you may have acid reflux or problems swallowing.

  • Sclerodactyly. As your skin thickens, it becomes stiff and shiny. You may find it hard to move your fingers. They can lock in a claw position. If the syndrome affects your face, you may have trouble opening your mouth.

  • Telangiectasia. The blood vessels under your skin may swell. They can cause small red spots to form. These often show up on the face and lips. 

You may also feel very tired (fatigued). Some people describe this tiredness as similar to that in people getting cancer treatment. Or similar to the fatigue caused by lupus or rheumatoid arthritis. 

Over time, CREST syndrome can cause problems with your internal organs, such as your heart and lungs. They may need to work harder. For instance, you may develop pulmonary hypertension. That’s high blood pressure in the arteries that carry blood to your lungs. 

Treatment for CREST syndrome 

Your healthcare provider will talk about treatments with you. He or she will need to find out how severe the diseases are. Then your provider will come up with treatment plan that’s right for you.

CREST syndrome can’t be cured. Treatments address the symptoms you have. They may include: 

  • Moisturizing lotions.These may help with dry, stiff skin.

  • Exercise and physical therapy. Moving more may help ease the pain and stiffness in your joints.

  • Antacids. These medicines can help ease acid reflux.

  • Penicillamine. This medicine may slow down the buildup of collagen in the body. But it does not work for everyone.

  • Blood pressure pills. These can treat pulmonary hypertension.

  • Calcium-channel blocking agents. These medicines can raise blood flow to your hands and feet to prevent Raynaud syndrome. Staying warm can also limit this symptom.

  • Laser treatment. It may reduce the red spots caused by swollen blood vessels. 

Possible complications of CREST syndrome 

  • Lung problems

  • Liver disease

  • Heart failure

  • Kidney failure

  • Dental problems

When to call your healthcare provider

Call your healthcare provider right away if you have any of these:

  • Fever of 100.4°F (38°C) or higher, or as directed by your healthcare provider

  • Pain that gets worse

  • Symptoms that don’t get better, or get worse

  • New symptoms


November 18, 2017


Brown TA, et al. Rheumatology In: Brown TA, ed. USMLE Step 1 Secrets. 3 ed. Philadelphia: Saunders; 2013:577-617., Neville BW, et al. Dermatologic Diseases. In: Neville BW, ed. Oral and Maxillofacial Pathology. 4 ed. Philadelphia: Elsevier; 2016:690-760., Overview of the Clinical Manifestations of Systemic Sclerosis (Scleroderma) in Adults. UpToDate., Overview of the treatment and prognosis of systemic sclerosis (scleroderma) in adults. UpToDate., Sinha AC. CREST Syndrome. In: Fleisher LA, ed. Essence of Anesthesia Practice. 3 ed. Phildelphia: Saunders; 2011:103.

Reviewed By:  

Blaivas, Allen J, DO,Fraser, Marianne, MSN, RN