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What Is CREST Syndrome?

Updated on September 8, 2012

CREST Syndrome

CREST syndrome is an acronym for 5 cardinal features of the condition namely:

C - Calcinosis

R - Raynaud's Phenomenon

E - Esophageal Dysmotility

S - Sclerodactyly

T - Telangiectasia

These 5 different signs and symptoms of the condition may sound totally foreign to a layman but essentially each of these 5 cardinal features refer to separate distinct features to help physicians make a diagnosis for this syndrome.

Below are the meanings for each of them in simpler language...

Calcinosis - calcification of soft tissues

Raynaud's Phenomenon - A phenomenon whereby your hands changes from looking pale, to blue and then to red in response to cold temperatures or emotional stress.

Esophageal Dysmotility - Abnormal movement in you food tube also known as the oesophagus resulting in symptoms of heartburn, reflux disease and difficulty swallowing.

Sclerodactylyl - This word means thickening and tightening of the skin of the fingers and toes

Talengiectasiae - Refers to the dilation of blood vessels close to the skin surfarce and mucous membranes.

CREST Syndrome is in fact a milder form of Scleroderma which is very much a systemic disease that affects multiple organs that include your skin, GI tract, lungs, heart and kidneys.

CREST Syndrome is in fact a milder form of Scleroderma...

Below are some famous people who have scleroderma...

and those directly involved in some charity to fight scleroderma.

Famous People With Scleroderma / Involved With Scleroderma - Jason Alexander - Seinfeld's George Costanza

Jason Alexander, the comedian, actor and Broadway singer with numerous nominations and awards under his sleeve is the spokesperson for the Scleroderma Foundation and has been one of their greatest supporters for a long time. He DID NOT PERSONALLY have scleroderma but was an avid supporter for the foundation as he has a sister affected by the disease.

Tylyn John - Playboy Playmate 1992 - Her Story of Scleroderma

Tylyn John may be a playboy playmate many years ago but she is now retired from all of that and working for various charitable organizations including the Scleroderma Foundation.

Tylyn John was diagnosed at the time just after ther son's birth in 1998. Her symptoms wasn't full blown at first but gradually progressed through time.

As with most cases of scleroderma, it started with extreme fatigue, Raynaud's in her fingers and later on tightening in her hands and forearms. It later progressed proximally to her upper arms and shoulders and then to her mouths and cheek. Finally it begin to involve the joints in her jaw, known as the temporomandibular joint which significantly reduced mouth opening and pain. Can you imagine experiencing all that?

Her symptoms wasn't recognized earlier on until later when she was diagnosed by one of America's foremost rheumatologist and was started on Minocycline which helped with her disease and soon all the symptoms of skin tightening, hardening and TMJ involvement disappeared over time.

Jean Racine - Olympic Bobsledding champion

Jean Racine also did not have scleroderma but lost her mum to scleroderma and is a supporter for the Scleroderma Fountation. Her mum died at the age of 47, which is very young IMO but suffered a lot from the disease before succumbing to it.

What Happens To People Who Get CREST Syndrome?

Signs and Symptoms to Observe

Although CREST Syndrome is quite a complex disease, there are stages of signs and symptoms to observe.

> A typical case of a patient with CREST Syndrome usually presents with Raynaud's phenomenon. This usually progresses for quite a while before patients start getting puffiness around their fingers and finally thickening of the fingers of the skin.

Patient's with Raynaud's phenomenon get changes that starts with a pale look on the skin (pallor), before turning blue (cyanosis) and then turning pink (rubor). Although a cold environment and stressful situations trigger Raynaud's, it can happen without any reason.

When patients suffer from Raynaud's, it can last for a few minutes up to a few hours.

> Sclerodactylyl is one of the next sequence of events that happens although they can happen together with Raynaud's. As mentioned earlier in this lens, sclerodactyly means thickening of the skin of the digits of the hands and feet.

Once this happens patients usually experience swelling of the fingers (oedema), morning stiffness. This swelling around the fingers or toes lasts for months before thickening comes in. When this happens, the skin will look tight and shiny. You will also experience itchiness. Over a few years, these skin changes progresses upwards (proximally) and involve the face and neck.

> Pain is not a common symptom unless calcinosis sets in. Calcinosis usually comes in relatively late at a time when the disease is relatively advanced.

Calcium deposits in the joints can cause intense pain and ulcerate revealing a chalky white substance... something you really don't want to experience or even see with your own eyes... trust me..

> Oesophageal motility is another relatively common CREST symptom. Here are some common problems associated with oesophageal motility:

a) Heartburn

b) Narrowing of the oesophagus (strictures). Strictures are really bad especially when eating and can cause intense pain.

c) Problems with food movement in the oesophagus leads to worsening of reflux disease and erosion of the lining of the oesophagus.

d) The nature of the disease also increases risk of bleeding in the gut. Telangiectasiae increases this risk.

> Fatigue, Joint pain and muscle weakness is another common symptom. Coughing is also common.

Of course, since CREST is a milder presentation of scleroderma (systemic sclerosis), patients do have symptoms and signs that will involve the lungs, heart and kidneys. However, if there is involvement of those organs, prognosis is poorer.

How To Diagnose CREST Syndrome?

I might as well say leave it to the physicians or doctors to make the diagnosis as it involves a rather complicated set of laboratory testing and imaging.

But for interests sake (hopefully after reading this far...), I shall give you an idea:

Lab Investigations are generally very specialized and are directed towards diagnosing connective disorders such as SLE, Sjogren's Syndrome, Rhuematoid Arthritis etc. Below are some of them:

  • ANA, Anti-topoisomerase Ab, Anticentromere antibodies
  • High White Cell Count, Anaemia, Low Haemoglobin
  • Barium Swallow
  • CXR
  • Lung Function Tests

There are plenty more tests but I wouldn't bore you with them.

LEAVE IT TO THE EXPERTS!

How to Support Scleroderma Research - This Includes Supporting Research For CREST Syndrome Too...

Scleroderma has no cure till this day...

Physicians can only give drugs that will bring it to remission.

CREST syndrome is a subset condition of scleroderma and so if you support any Scleroderma organisations, you support research that will find a CURE for it!

Donate to the Scleroderma Foundation by clicking on the picture ABOVE.

Scleroderma Research Foundation

I found this very moving video on scleroderma patients. Check it out.

You might want to support this organization by donating here.

Increase Scleroderma Awareness!

Support scleroderma research throught these awareness products!

Walking Aids To Help the Elderly

Whether or not a member of your family or friend has scleroderma, rheumatoid arthritis or any other form of disease, walking aids will help them with their mobility.

You may need to consult a doctor or a physiotherapist before proceeding with buying them. If you think it is a good idea getting them, consider it!

How to Treat CREST Syndrome?

CREST Syndrome is a milder form of scleroderma or widespread systemic scleroderma which means that the condition is less serious. Although it is not as serious as systemic scleroderma, there is NO KNOWN CURE for CREST Syndrome.

But that is not to say that there is not treatment to bring the disease into remission.

Here are some recommendations by experts and physicians:

1) For Raynaud's Phenomenon - It can be treated with calcium channel blockers. Drugs like nifedipine have some proven benefit for Raynaud's phenomonon. Other options include GTN or glyceryl trinitrate, the same drug used for people with angina ( a pre-indicatior of MI ). There are other options available like blood pressure drugs but they still requiring further studies.

Other options include non medical therapy such as wearing gloves and wearing adequate clothing/covering to prevent exacerbation of Raynaud's Phenomenon. Staying indoors is one of the easiest thing you can do.

2) Problems with oesphageal movement - Preventive measures such as weight loss, reduction in caffeine and alcohol intake reduce symptoms of reflux and heartburn. Antacids and PPI (used in the treatment of stomach ulcers) also help.

3)Treatment of Sclerodactyly is not curative. It only reduces swelling, thickening and also reversal of skin changes. Steroids and NSAIDS are used. More research is being carried out on other agents like D- Penicillamine.

4)Psychosocial aspects of the disease also need to be addressed as CREST syndrome is a debilitating disease and can lead to depression, low mood, anxiety and sometimes suicidal ideas. Counselling, social support and family support are therefore paramount in patients suffering from this condition

5)Physiotherapy. You may not get access to this easily but basically, physiotherapist teaches patients exercises to help strengthen muscles to compensate for joint weakness. Stretching exercises are also taught which are important in joint mobility.

The Bottomline is...

Treatment improves symptoms of pain and can bring the disease into remission but...

it will NOT CURE CREST syndrome....

Alternative Treatments for CREST Syndrome / Scleroderma - Is There One?

As mentioned before, there isn't any cure for CREST syndrome. Drugs used to treat CREST are not effective in everyone and carry a high risk of side effects. Steroids may be used in many other conditions like asthma and other autoimmune disease but it really just reduces inflammation and masks the disease.

Alternative remedies to this condition may not be supported by a lot of evidence in the literature but many would prefer alternative therapies that have less side effects compared to traditional drug therapy for the condition.

Here are some natural treatments that may have some benefit. Most of these compounds are antioxidant in nature.

  • Vitamin E
  • has been used to treat scleroderma but not CREST syndrome. Its antioxidant properties may have a role to play in treating Raynaud's phenomenon, skin changes in the condition and calcinosis
  • Vitamin D is another treatment that people used in the early 20th century but due to its side effects, it has not been considered as an alternative option until now. A few studies carried out showed some benefits but not good enough for long term therapy. They are side effects with high doses of Vitamin D such as calcium in urine and blood.

  • Evening Primrose Oil has been effective in some people with the disease. Notable difference include healing of ulcers, improved skin texture and reduced occurrence of Raynaud's phenomenon.

Other potential alternative treatments include S-Adenosylmethionine and Soybean extract but I'm not sure how effective they are against CREST Syndrome or Scleroderma...

Did This Lens Teach You Much More about CREST Syndrome?

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What Are Your Thoughts on Scleroderma?

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    • profile image

      anonymous 3 years ago

      I have CREST syndrome and would love to get involved in any studies to help with research if anyone knows how to go about this it would be appreciated...one thing I did not see mentioned was fish oil I take this every day along with evening primrose oil for good vein help. would love any suggestions on things people have tried for Raynauds which I suffer from quite badly.

    • profile image

      anonymous 3 years ago

      I have CREST syndrome and would love to get involved in any studies to help with research if anyone knows how to go about this it would be appreciated...one thing I did not see mentioned was fish oil I take this every day along with evening primrose oil for good vein help. would love any suggestions on things people have tried for Raynauds which I suffer from quite badly.

    • profile image

      anonymous 4 years ago

      I have crest disease, I am searching for cure. I also have dry eyes and mouth. This information helpe a lit. Thank you

    • profile image

      anonymous 4 years ago

      I return visit and recommend for other articles

    • gregs411 profile image

      gregs411 4 years ago

      Have never heard of this disease before. A very nice overview. Thanks.

    • bwet profile image
      Author

      bwet 4 years ago

      @BLemley: Hi BLemley, it is a rare but horrible disease for those who have it. thanks for the blessing!

    • BLemley profile image

      Beverly Lemley 4 years ago from Raleigh, NC

      My heart goes out to the victims of this horrible disease. Thanks for bringing about awareness ~ SquidAngel blessed! B : )

    • bwet profile image
      Author

      bwet 5 years ago

      @JohnTannahill: Thanks John for stopping by! I hope this lens has taught you all you need to know about the condition.

    • JohnTannahill profile image

      John Tannahill 5 years ago from Somewhere in England

      Very informative - this is all new to me.

    • bwet profile image
      Author

      bwet 5 years ago

      @Alethia LM: Hey Alethia, thanks for stopping by and liking my lens.

    • bwet profile image
      Author

      bwet 5 years ago

      @JoshK47: Thx Josh for the blessing! Appreciate it :D It is a pretty rare condition and i hope its good info for you

    • Alethia LM profile image

      Alethia LM 5 years ago

      I had no idea about Scleroderma until this lens. Thank you very much for sharing!

    • profile image

      JoshK47 5 years ago

      Quite an informative lens - I didn't know much about scleroderma until I read this. Thanks for sharing - blessed by a SquidAngel!