What You Need to Know About Crest Syndrome: Symptoms, Causes, Risk

What is CREST Syndrome? 

CREST syndrome is a type of autoimmune condition that leads to stiff, hardened skin and connective tissues. Specifically, CREST syndrome is a subtype of the autoimmune disease scleroderma.  

CREST syndrome stands for: calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia. The 5 symptoms making up the CREST acronym are defining symptoms of CREST syndrome. 

What is Calcinosis? 

Calcinosis is the collection of calcium in muscles, tendons, or under the skin. These calcium deposits form hard, visible lumps. Sometimes, these lumps may be painful,  lead to ulcers or, affect the range of motion or movement.  

What is Raynaud’s Phenomenon? 

Reynaud’s phenomenon is a condition that leads to limited blood flow to the extremities, specifically fingers, toes, ears, knees, or nose. This phenomenon may occur on it’s own, or may be associated with an autoimmune disease.  

Common features of Raynaud’s are pale discolored skin, tingling, coldness, or throbbing in the affected areas.  

What is Esophageal Dysfunction? 

The esophagus is the organ in the body that connects the pharynx (throat) to the stomach. Muscles in the esophagus work to move food and liquids from your mouth into the stomach. When the esophagus dysfunctions, it can cause a variety of symptoms: 

  • Difficulty swallowing 
  • Sore throat or chronic cough 
  • Regurgitation 
  • Chest pain, pressure, or heartburn 
  • Weight loss 

Esophageal dysfunction may be connected to a number of disorders, but is a common feature in people with CREST syndrome.  

What is Sclerodactyly? 

Sclerodactyly is the tightening and thickening of the skin, a common feature of scleroderma and CREST syndrome.  

Sclerodactyly may start as swelling (commonly in the fingers and toes) and progress when the skin becomes hard or shiny. In severe cases, it may cause the curling or disfiguration of the fingers or toes. 

What is Telangiectasia? 

Telangiectasia is more commonly known as spider veins. The natural aging process, sun exposure, or genetics may cause small blood vessels to dilate. However, if spider veins occur along with other symptoms associated with CREST syndrome, it may be caused by an autoimmune condition, such as lupus or scleroderma. 

Telangiectasia is common on the legs, upper chest, neck, and face. They may appear as blue, purple, or reddish weaving “threads” or fine lines. 

Scleroderma and CREST Syndrome 

CREST syndrome is a subtype of scleroderma. Specifically, it’s in a group of scleroderma subgroups called limited scleroderma. Limited scleroderma is the most common form of scleroderma. Limited scleroderma is more localized, meaning it typically only affects certain areas of the body like the fingers/hands, or face.  

In rarer cases, CREST syndrome may lead to pulmonary hypertension, which can be life-threatening if not treated and monitored.  

There are many types of scleroderma, which may make it difficult to diagnose. Your rheumatologist may be able to determine the type of scleroderma you have. They will likely conduct a physical examination, review your symptoms, and order a variety of tests to rule out other causes. 

Crest Syndrome Symptoms 

The most common symptoms of CREST syndrome are symptoms associated with the 5 key features of the disease: calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia: 

  • Bumps under the skin, specifically around the fingers or tones 
  • Ulcers or sores 
  • Swelling, tightening, or thickening around the extremeities 
  • Discoloration, tingling, or numbness of the extremities 
  • Heartburn 
  • Difficulty swallowing 
  • Sore throat 
  • Weight loss 
  • Bloating 
  • Constipation or diarrhea 
  • Joint Pain  
  • Spider veins 
  • Heart failure (rare cases) 
  • Kidney disease (rare cases) 

CREST Syndrome Treatment 

Treatment for CREST syndrome is typically a combination of lifestyle changes and medications to help manage the individual symptoms and reduce inflammation. 

CREST Syndrome Medications 

Pain Relievers: NSAIDs (like ibuprofen or Naproxen) can help ease mild joint pain or reduce swelling in the extremities. 

Immunosuppressants: Medications like methotrexate, mycophenolate mofetil, and cyclosporine suppress the immune system to help control your body’s inflammatory response. 

Individual Symptom Treatments: 

For GI Symptoms (esophageal dysfunction): Proton Pump Inhibitors or calcium channel blockers can help relieve heartburn and indigestion. 

For Reynaud’s Phenomenon: Vasodilators or calcium channel blockers can help relax and open the blood vessels. 

For Telangiectasia (spider veins): Spider veins don’t pose a health risk and therefore don’t require treatment. However, laser therapy can help remove the appearance of spider veins. Electrodesiccation may also be used, which involves the insertion of a small needle into the vein to deliver an electrical current.  

Physical Therapy for CREST Syndrome 

Apart from regular exercise to help improve blood circulation, physical therapy may be an option for some patients with CREST syndrome to help manage their symptoms.  

Your CREST syndrome specialist or rheumatologist will work with you to identify the best treatment for you. They may be able to recommend certain stretches or exercises to help alleviate pain or issues with mobility.  

To learn more about our CREST syndrome treatment specialists in Arizona or Oklahoma, send us a message.  

Oklahoma Contact Information

Phone

Edmond: (405) 260-8605

Fax

(405) 369-9310

Arizona Contact Information

Phone

Gilbert: (480) 494-2770
Casa Grande: (520) 557-5660

Fax

(480) 494-2771

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