CREST Syndrome

CREST syndrome is a type of scleroderma that causes fewer symptoms compared to other types. It’s also known as limited scleroderma. CREST is an acronym for the common symptoms it causes.

Scleroderma is a chronic disease that causes your body to make too much collagen. This changes your body’s tissues, making them thick and fibrous, like scar tissue. Scleroderma means scarring or thickening of your skin. But this thickening and hardening can also affect internal tissues and organs. Another name for scleroderma is systemic sclerosis, which means scarring throughout your body.

CREST syndrome (limited systemic sclerosis) affects a variety of tissues in your body. But it doesn’t affect as many internal tissues as other types can. Experts believe this is because it progresses very slowly.

Symptoms of CREST syndrome

CREST is an acronym for the main symptoms the syndrome causes. CREST stands for:

• Calcinosis cutis: This means calcium deposits form in your skin. They look like firm, white or yellow bumps and often appear on your joints, fingertips and face.
• Raynaud’s phenomenon: This means you have occasional spasms in your small blood vessels in places like your fingers and toes. They’re painful and make your digits turn blue.
• Esophageal dysmotility: This means the muscles in your esophagus don’t move as effectively as they should. It can cause chronic acid reflux and difficulty swallowing.
• Sclerodactyly: This means scarring of the skin on your fingers. The skin can become so tight and rigid that it causes contractures in your fingers, making them hard to bend.
• Telangiectasias: These are dilated (widened) capillaries under your skin that appear as red or purple marks. They might look like dots, spider angiomas or spider veins.

CREST syndrome causes

CREST syndrome, like other forms of scleroderma, is an autoimmune disease. This means your immune system causes it by mistake. Your immune system creates antibodies that attack certain cells in your body, the way it would normally attack a germ. This attack causes inflammation in your tissues. Chronic inflammation leads to scarring (collagen production). Scarring produces excess calcium as a byproduct.

Complications of CREST syndrome

CREST syndrome (limited scleroderma) doesn’t affect as many organs or cause as many complications as diffuse systemic sclerosis can. It progresses slowly, and the symptoms are often manageable with treatment. But severe symptoms can cause complications, like contracted fingers, swallowing difficulties or painful sores in your skin. Life-threatening complications are rare, but possible. They include:

• Gangrene: Raynaud’s syndrome cuts off blood supply to your fingers and toes, usually briefly. But if this blood loss is severe or prolonged, it can cause tissue death and lead to amputation.
• Pulmonary hypertension: If CREST syndrome spreads to your pulmonary arteries, it can cause them to scar and narrow. This restricts their blood flow and strains your heart.

How is CREST syndrome diagnosed?

To diagnose CREST syndrome, a healthcare provider will give you a thorough exam. They’ll look for at least three of the five symptoms named in the acronym. Any of these symptoms can also occur with other forms of scleroderma. But the combination of these specific symptoms appearing together suggests CREST syndrome. Blood tests for CREST-related antibodies can also help to make the diagnosis.

How is CREST syndrome treated?

Treatment for CREST syndrome primarily focuses on treating the individual symptoms. Like other autoimmune diseases, CREST syndrome has no cure. Medications that suppress your immune system (immunosuppressants) may slow the disease down and reduce its effects. But there are side effects to taking immunosuppressants, and they don’t always help enough to make taking them worthwhile.

Treating CREST syndrome means:

• Treating calcinosis: Calcium deposits in your skin can become painful and may break the skin, leading to infection. You may need topical antibiotics or pain relievers or, in some cases, surgery to remove them. Your provider might also suggest oral medications for calcinosis.
• Treating Raynaud’s phenomenon: Calcium channel blockers can help your small blood vessels relax. This can prevent the spasms and constriction that cause Raynaud’s phenomenon. Since cold triggers these spasms, your provider will also recommend ways to keep your digits warm.
• Treating esophageal disorders: Medications called proton pump inhibitors are the standard treatment for chronic acid reflux. They reduce the acid in your stomach to protect your esophagus. For swallowing difficulties, your provider might recommend esophageal dilation.
• Treating sclerodactyly: Your provider might suggest physical therapy for your hands to keep them flexible. UV light therapy may help reduce inflammation and related symptoms in your skin, like itching or pain. Therapeutic lotions or topical steroid creams might also help.
• Treating telangiectasias: Telangiectasias are usually only a cosmetic issue. You can treat them with laser therapy or sclerotherapy. But sometimes, the dilated blood vessels can break and bleed. This could require further treatment, like medications or procedures to stop the bleeding.

What can I expect if I have CREST syndrome?

CREST syndrome can certainly affect your quality of life. But it isn’t as life-threatening as diffuse scleroderma. In general, how much of your skin is involved is a good indicator of how much the disease will affect you. If it mostly affects your fingers, which is typical of CREST syndrome, your outlook is good. The most serious risk is pulmonary hypertension, which isn’t common and usually takes decades to develop.

Is there anything I can do to feel better?

Living with CREST syndrome means managing a variety of symptoms in different parts of your body at once. It can feel a bit like a game of whack-a-mole. Some consistent self-care can help maintain a base level of health and comfort. For example, your healthcare provider might advise that you:

• Reduce acid reflux: You can do this by reducing fat in your eating plan, eating dinner earlier and elevating your head when you sleep.
• Reduce Raynaud’s syndrome attacks: Staying active, managing stress and keeping your hands and feet warm may help.
• Quit smoking: This improves your overall health and reduces your risk of complications from CREST syndrome.
• Monitor your health: Notice and report any new symptoms that appear to help your provider catch complications sooner rather than later.

When should I seek urgent care?

Call or see your provider right away if you experience:

• Sudden chest pain
• Frequent dizziness or faintness
• Labored breathing
• Blood in your stool