The Difference Between Lupus and CREST Syndrome

Lupus and CREST syndrome are both autoimmune disorders. Getting an accurate diagnosis can be hard because the 2 have several overlapping symptoms. In this article, we will take a look at what CREST syndrome is and how it is different from lupus.

What is CREST syndrome?

Scleroderma is a rare autoimmune connective tissue disorder. It is recognized by abnormal thickening of the skin.¹ CREST syndrome is a type of scleroderma called limited scleroderma.² CREST is an acronym for symptoms of the disease.^1,2

• C stands for calcinosis. This means calcium deposits form on the fingers or under the skin.
• R stands for Raynaud's phenomenon. In Raynaud’s, blood vessels in the fingers and toes spasm when exposed to stress or cold. This means the fingers and toes cannot stay warm. They may become painful and/or very pale.
• E stands for esophageal dysmotility. This means the esophagus does not function correctly. It can lead to trouble swallowing and cause heartburn.
• S stands for sclerodactyly. This means the skin and other connective tissue tighten. It can cause fingers to bend.
• T stands for telangiectasia. These are enlarged blood vessels, usually appearing as red spots on the skin.

People living with lupus and CREST syndrome experience the diseases differently. Symptoms of the same disorder can also appear differently in different people.²

Lupus and CREST syndrome

Much like lupus, scleroderma is an immune system disorder.^1,2 People living with autoimmune disorders may have more than one. When this occurs, the disorders are called overlapping or crossover diseases.^1,2 About 20 percent of people living with scleroderma also have lupus.^1,2

CREST syndrome and lupus symptoms

Lupus has many different symptoms, but some common symptoms are:^1,3

• Joint pain
• Muscle pain
• Extreme fatigue
• Skin rash
• Weakness

Overlapping symptoms common to lupus and CREST syndrome include:^1,2

• Raynaud’s
• Problems swallowing or digestive issues
• Sjogren’s syndrome, which can cause dry mouth, dry eyes, dry skin, pain, and fatigue
• Painful joints
• Fatigue

CREST syndrome symptoms

Every person with scleroderma is different and has a different pattern of symptoms. People with CREST syndrome have at least 2 of these main symptoms:^1,2

• Bumps under the skin
• Raynaud’s
• Trouble swallowing or digestion issues
• Tight or hard skin
• Red spots or lines on the skin

CREST syndrome causes

The exact causes of lupus and CREST syndrome are unknown. Doctors believe a combination of factors can increase a person’s risk for developing either condition.

Lupus risk factors

Risk factors for lupus include:⁴

• Genetics
• Environment
• Hormones
• Certain medicines, viruses, and infections

Doctors and researchers know about dozens of genetic variants linked to lupus. These genes can affect who gets lupus and how severe it is.⁴

CREST syndrome risk factors

Risk factors for CREST syndrome include:^1,5

• Sex
• Age
• Race
• Genetics
• Exposure to toxins

Women are more likely to develop CREST syndrome than men. It is more common in people between the ages of 30 and 50.^1,5 More severe cases of CREST syndrome are found in Black and Native American people.⁵ If a family member has an autoimmune disease, you also have a higher risk of developing CREST syndrome.⁵