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CREST Syndrome
pathology perspectives

By Ruth Werner

Originally published in Massage Bodywork magazine, November/December 2009. Copyright 2009. Associated Bodywork and Massage Professionals. All rights reserved.

CREST syndrome. Sounds like a term referring to dental hygiene, doesn't it? In fact, it is an acronym for a group of signs and symptoms that are associated with one form of scleroderma, an autoimmune disease.

Scleroderma isn't particularly common; about 300,000 people in the United States have been diagnosed with it. But exploring this disease reveals some things about chronic inflammation, immune system dysfunction, and the nature of connective tissue that might intrigue massage therapists.


What is Scleroderma?

Scleroderma (literally, "hard skin") refers to a group of autoimmune diseases with a particular outcome: whatever the tissue attacked, the body responds by investing the nearby areas with deposits of collagen and other connective tissue extracellular matrix, thus causing the area to become destructively hardened or scarred. Most forms of scleroderma affect the skin distal to the elbows and knees, but it may also affect the skin of the trunk, face, or neck. Areas of involvement can be rounded or streaked. In some cases, these deposits of collagen can also affect the internal organs (especially the lungs, gastrointestinal tract, and kidneys), and the rarest form of the disease affects the organs only, with no skin involvement.

The etiology of scleroderma is not at all well understood. Gender clearly has a role: it occurs in women about four times more frequently than in men. (Interestingly, the Choctaw women of Oklahoma have the highest incidence of scleroderma.) Most cases are diagnosed between ages 30 and 50. It seems to arise most often in families where certain other autoimmune diseases occur, specifically lupus, rheumatoid arthritis, and Hashimoto's thyroiditis. It seems clear that the combination of genetic susceptibility and environmental toxic exposures (silica, polyvinyl chloride, benzene, and trichloroethylene are at the top of the list) provide powerful triggers for the immune system to malfunction, but in most cases the exact causes of this disease are unknown.1

When scleroderma occurs, the immune system appears to attack the lining, or intima, of small- and medium-sized blood vessels. As part of the inflammatory response, other immune system cells collect in the affected areas: macrophages, eosinophils, basophils, antibody producing B cells, and mast cells (all associated with pro-inflammatory activity). These cells secrete pro-inflammatory chemicals, while regulator T cells, which would ordinarily suppress the inflammatory response, are conspicuously absent.

The concentration and heightened activity of these white blood cells appear to be early indicators of the disease process. Even in situations without autoimmune dysfunction, it is readily seen that chronic inflammation leads to the development of excessive scar tissue. In scleroderma, that process seems to run completely amok. Fibroblasts (connective tissue producing cells) that live in the skin and small blood vessels are activated. These cells begin to produce enormous amounts of collagen and other connective tissue matrix.

This material is then deposited in the interstitial spaces and in the intima of the affected blood vessels. This usually happens in the skin, but other tissues may be affected as well. Further, the disruption of the innermost layer of small blood vessels can impede blood flow to vital tissues--for instance, the motor nerves that supply the digestive tract.

From this description, it is clear that scleroderma is a serious, even life-threatening disease. The good news is that it doesn't always occur in the worst possible form, and for many people it is quite mild. Some patients find that their disease progresses for several years, then stabilizes for several years, and then mysteriously resolves, never to occur again.


Types of Scleroderma

Scleroderma is classified into two main groups, each with subtypes.

Localized Scleroderma

This affects the skin only and is not associated with organ dysfunction.

Morphea Scleroderma

Oval-shaped patches appear on the skin of the extremities, although the trunk is occasionally involved. These are often pale in the middle and purplish around the edges. These areas are hard and inelastic, and the skin may appear shiny and thickened. Ulcerations may occur as well.

Linear Scleroderma

This form involves streaks of hardened skin, often on the face or neck. A particular facial lesion is sometimes called coup de sabre or sabre cut for its scar-like appearance on the face.

Systemic

This usually affects the skin, but internal organs are also sites of collagen deposition.

Limited Cutaneous Systemic Scleroderma

This condition mostly involves the skin, but the gastrointestinal tract is also frequently involved. The acronym CREST, described below, was coined for the symptomatic profile seen with this version of scleroderma.

Diffuse Cutaneous Systemic Scleroderma

This can involve the skin and all the internal organs, but frequently focuses on the lungs, heart, and kidneys. This is a severely threatening form of the disease, investing vital organs with deposits of useless scar tissue that interferes with their function.

Sine Scleroderma

Sine means without. In this rare form the skin is spared, but the internal organs are invested with connective tissue that interferes with their ability to function normally.


CREST Syndrome/ABCDCREST Syndrome

CREST syndrome, discussed previously, is an acronym for the symptomatic profile that is most commonly seen with limited cutaneous systemic scleroderma. It stands for calcinosis; Raynaud phenomenon; esophageal dysmobility; sclerodactyly; and telangiectasias. In 2004, a group of specialists convened to gather information about cutaneous systemic scleroderma, and revised the acronym to this: ABCDCREST. They found that if a patient has any three or more of these nine signs, the chance of their having this form of scleroderma is about 99 percent, making this a far more accurate test than many of the intrusive procedures people typically go through on their way to a definitive diagnosis.

A--Autoantibodies

This refers to the presence of distinguishable antibodies in blood tests that indicate a tendency toward autoimmune disease.

B--Bibasilar Pulmonary Fibrosis

This indicates the deposition of collagen fibers in the lungs, which reduces lung function and increases the risk of infection. This may be due to the disease itself, but it may also be the resulting irritation from aspirated food and liquid due to esophageal reflux.

C--Contractures of Digital Joints

Contractures are permanently shortened muscles due, in this case, to the loss of function at the nearby joints. This comes about because the skin on the hands is so tight the fingers can't move.

D--Dermal Thickening Proximal to Wrists

The hands of a patient with scleroderma are often involved; when the thickened, hardened skin occurs proximal to the wrists (i.e., on the trunk, neck or face) this is a more definitive sign of cutaneous systemic disease.

C--Calcinosis Cutis

This refers to small calcium deposits in the skin. These can be too tiny to recognize, or of significant size, causing pain, further inflammation, and ulcerations.

R--Raynaud's Phenomenon

This describes periodic bouts of extreme vascular contractions in the hands or feet. The skin turns white with lack of blood flow, then blue with longer-term oxygen deprivation, and finally red as the blood flow is returned. Raynaud's phenomenon is not synonymous with Raynaud's disease, which is a free-standing condition often related to cold exposure and overuse. Raynaud's phenomenon, by contrast, is always associated with autoimmune disease and tends to be more severe and carry the risk of complications like tissue damage and even gangrene.

E--Esophageal Dysmobility

A consistent experience of patients with cutaneous systemic scleroderma is problems with digestion. These challenges typically start at the proximal end of the digestive tract with esophageal dysfunction--it becomes difficult to pass food through to the stomach. It often feels lodged or stuck in the esophagus, and it may require vomiting to clear the passage. This happens because blood vessels that supply the motor nerves to the gastrointestinal tract are impaired, and the result is general peristaltic weakness. Chronic reflux (along with heartburn and the risk of esophageal cancer from ongoing irritation) is one possibility, but impaired motor nerve supply can affect the small and large intestines with generally poor smooth muscle tone and interrupted peristaltic function.

S--Sclerodactyly

This means hardening of the fingers. One of the hallmarks of scleroderma is patches of hardened, shiny skin, usually on the hands. The skin can become so tight that the fingers can no longer move, leading to the contractures discussed above.

T--Telangiectasias

These are clusters of tiny dilated blood vessels. They are commonly called spider veins. On the skin, they are harmless, and often appear close to larger varicose veins. In patients with scleroderma, they appear on the face, especially around the mouth, and on the hands. These are unusual areas for these skin markings. GI scopes may also reveal telangiectasias in the lining of the stomach of scleroderma patients as well. This is a more serious problem, because in this location they may rupture and bleed, leading to serious anemia.


Other Signs, Symptoms, and Complications

Any type of systemic scleroderma carries a long list of possible complications. Again, not every patient is at risk for these problems, but part of good care is being watchful in order to anticipate and treat these challenges as they arise. Lung damage (including an increased risk of lung cancer) is a threat, as the delicate tissues are invested with scar tissue. This can cause pulmonary hypertension and right-sided heart failure as the heart tries to push blood through the obstructed and resistant pulmonary circuit. Sjogren's syndrome describes pathologically dry eyes and salivary glands that can lead to both eye and mouth infections. When the skin on the face is affected, tooth decay and infection is a significant issue, because the patients can't open their mouths wide enough to brush their teeth. Joint pain is common with scleroderma patients, although the arthritis is not usually erosive. Excessive scar tissue may bind up peripheral nerves, leading to entrapment symptoms. Finally, the kidneys may become a target of the inflammatory onslaught, leading to the accumulation of scar tissue and loss of function. About half of the deaths directly due to scleroderma are related to renal failure.


Treatment for Scleroderma

Because this disease takes a unique path and time line with each patient, no universally applicable treatment protocol for scleroderma has been developed. The highest priority is to manage the symptoms and complications so that they create a minimum of destructive tissue changes. These strategies can include drugs to manage acid reflux and improve digestive motility; lotions and physical therapy to keep skin as stretchy as possible; and careful and thorough maintenance of tooth, heart, lung, and kidney health. Some of the disease-modifying immune system drugs that are highly successful for treating other autoimmune diseases are used in this context, but managing complications appears to take precedence.


Massage?

A client with scleroderma who has developed any of the organ-related complications discussed above, especially involving heart, lung, or kidney function, will require adjustments in treatment approaches to stay within the client's ability to adapt to the changes massage requires.

Interestingly, a few small-scale research studies have included massage as a treatment option for scleroderma patients.

One of them studied acupressure to indirectly affect esophageal dysfunction. The finding was that it is possible to change esophageal mobility with this intervention.2 Another study asked if skin mobilization massage, along with exercise and other therapies, was better for longtime function than home-based exercise alone. Not surprisingly, it was.3 And finally, one study followed what kinds of CAM therapies scleroderma patients pursued in addition to their conventional medical care. Massage therapy was a popular choice, and the authors postulated that stress management was a leading incentive in that pursuit.4

No firm guidelines for massage and scleroderma have been developed, but some variables clearly provide boundaries for modality choices. Because the quality of the skin is often affected, a therapist might have to make significant adjustments to a treatment protocol. These might include special focus on improving mobility, avoiding ulcerations, or respecting the fragility of atrophied areas.

Because scleroderma is a potentially serious disease that is not well understood, any massage therapist who has a client with this condition would do well to communicate with the rest of the health-care team to get the best guidance about possible risks and benefits of bodywork. This is an occasion to blaze a trail: I hope anyone with this opportunity would carefully document the experience and share it with the rest of us.

Ruth Werner is a writer and NCBTMB-approved provider of continuing education. She wrote A Massage Therapist's Guide to Pathology (Lippincott Williams Wilkins, 2009), now in its fourth edition, which is used in massage schools worldwide. Her new book, Disease Handbook for Massage Therapists, is now available from Lippincott Williams Wilkins. Werner can be reached at www.ruthwerner.com or wernerworkshops@ruthwerner.com.

NOTES

1. "Limited scleroderma (CREST syndrome)," Mayo Foundation for Medical Education and Research (MFMER). Available at mayoclinic.com/health/crest-syndrome/ds00580 (accessed October 2009).
2. D. Wollaston et al., "Patients with Systemic Sclerosis Have Persistent Alterations in Gastric Myoelectrical Activity with Acupressure to Neiguan Point PC6," The Journal of Rheumatology 32, no. 3 (2005).
3. S.M. Bongi et al., "Efficacy of Connective Tissue Massage and Mc Mennell Joint Manipulation in the Rehabilitative Treatment of the Hands in Systemic Sclerosis," Clinical Rheumatology 28, no. 10 (October 2009): 1167-73.
4. K. Hui et al., "Scleroderma, Stress and CAM Utilization," Evidence-Based Complementary and Alternative Medicine, 2007. Available at http://ecam.oxfordjournals.org/cgi/content/short/nem142v1?rss=1 (accessed October 2009).




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