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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Limited scleroderma


Other Names for this Disease

  • Calcinosis, Raynaud's phenomenon, Esophageal dismobility, Sclerodactyly, Telangiectasia syndrome
  • Calcinosis-Raynaud phenomenon-sclerodactyly-telangiectasia
  • CREST syndrome
  • Limited cutaneous systemic scleroderma
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Treatment

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How might CREST syndrome be treated?

Unfortunately, CREST syndrome has no known cure. The condition carries both physical and psychological consequences, so a holistic approach to management should be taken.[1] Treatment generally focuses on relieving signs and symptoms and preventing complications.[2] Heartburn may be relieved by antacid medications that reduce the production of stomach acid. Medications that open small blood vessels and increase circulation may help relieve Raynaud's symptoms and reduce increased pressure in the arteries between the heart and lungs. Drugs that suppress the immune system have shown promise in preventing interstitial lung disease (a condition in which excess collagen collects in the tissue between the lungs' air sacs) in some people with CREST syndrome. To prevent loss of mobility, stretching exercises for the finger joints are important. A physical therapist can also show affected individuals some facial exercises that may help keep the face and mouth flexible. If CREST syndrome is making it difficult to perform daily tasks, an occupational therapist can help individuals learn new ways of doing things. For example, special toothbrushes and flossing devices can make it easier to care for the teeth. Surgery may be necessary for some affected individuals. Large or painful calcium deposits sometimes need to be surgically removed, and amputation of fingertips may be necessary if skin ulcers progress to gangrene.[2] Depression affects approximately 45% of patients with systemic sclerosis and 64% also develop anxiety, so early assessment and treatment of these psychological issues is recommended.[1]

For pain management, studies have shown that oxycodone is effective and safe for pain due to severe skin ulcers, while topical lidocaine helps reduce pain of digital ulcers in individuals with systemic scleroderma.[3][4]
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There are also some lifestyle changes and home remedies that may be helpful for some individuals with CREST syndrome. To reduce Raynaud's symptoms, individuals may consider wearing gloves or mittens outdoors when the weather is cool, and indoors when reaching into the freezer, for example. To maintain the body's core temperature, individuals may dress in layers and wear a hat or scarf, thermal socks, and well-fitting boots or shoes that don't cut off  the circulation. Individuals who smoke should talk to their doctor about the best ways to quit. Nicotine constricts the blood vessels, making Raynaud's phenomenon worse. Individuals who have difficulty swallowing may consider choosing soft, moist foods and chewing food well. To minimize acid reflux individuals may eat small, frequent meals; avoid spicy or fatty foods, chocolate, caffeine, and alcohol; and avoid exercising immediately before or after eating. Sitting upright for a couple of hours after a meal may also help. To help keep skin soft, individuals may avoid harsh soaps and detergents, while choosing gentle skin cleansers and bath gels with added moisturizers. Individuals may also consider bathing less frequently and taking brief baths and showers, using warm rather than hot water. Moisture levels in the home may be improved by using a humidifier to ease skin and breathing symptoms.[2]

For additional information about how CREST syndrome may be treated, the following article from eMedicine may be helpful:  http://emedicine.medscape.com/article/1064663-treatment#showall

The information provided here is for general educational purposes only. Individuals interested in learning about specific treatment options for themselves or family members should speak with their healthcare provider.
Last updated: 8/11/2011

References
  1. Jeanie C Yoon. CREST syndrome. eMedicine. September 22, 2009; http://emedicine.medscape.com/article/1064663-overview. Accessed 8/11/2011.
  2. Limited scleroderma (CREST syndrome). Mayo Clinic. June 2, 2011; http://www.mayoclinic.com/health/crest-syndrome/DS00580. Accessed 8/10/2011.
  3. Giuggioli D, Manfredi A, Colaci M, Ferri C. Oxycodone in the long-term treatment of chronic pain related to scleroderma skin ulcers. Pain Medicine. October 2010; 11(10):1500-1503. http://www.ncbi.nlm.nih.gov/pubmed/20456083. Accessed 8/11/2011.
  4. Ozgocmen S, Kaya A, Coskun BK. Topical lidocaine helps reduce pain of digital ulcers in systemic sclerosis (scleroderma). Clinical Rheumatology. May 2006; 25(3):378-379. http://www.ncbi.nlm.nih.gov/pubmed/16211339. Accessed 8/11/2011.


Clinical Trials & Research for this Disease

  • The Centers for Mendelian Genomics program is working to discover the causes of rare genetic disorders. For more information about applying to the research study, please visit their website.
  • ClinicalTrials.gov lists trials that are studying or have studied Limited scleroderma. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
  • The Scleroderma Clinical Trials Consortium is an international organization of scleroderma clinical researchers. The consortium Web site contains a listing of active scleroderma trials, past copies of the Scleroderma Care and Research journal, and a tool for finding your nearest member institution. 
Other Names for this Disease
  • Calcinosis, Raynaud's phenomenon, Esophageal dismobility, Sclerodactyly, Telangiectasia syndrome
  • Calcinosis-Raynaud phenomenon-sclerodactyly-telangiectasia
  • CREST syndrome
  • Limited cutaneous systemic scleroderma
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.