Your Questions Answered! CREST Syndrome

Originally published in "Scleroderma Voice," 2004 #4

QUESTION:
I was recently diagnosed with CREST Syndrome and am receiving some conflicting information about this disease. Is CREST Syndrome the same as scleroderma?

ANSWER:

CREST Syndrome, as it was known for many years, is one of three forms of systemic scleroderma: diffuse cutaneous systemic sclerosis, limited cutaneous systemic sclerosis, and a rare form of the disease known as systemic sclerosis sine scleroderma. Each will be defined in turn. CREST is an acronym that was created to describe the hallmarks of the disease by its most visible features. The letters represent the following: C-calcinosis, R-raynauds, E-esophageal dysfunction, S-sclerodactyly and T-telangectasias. In more recent years, as understanding of scleroderma has increased, it became apparent there was more to CREST Syndrome than the mere external manifestations of the disease. For this reason, the rheumatology community has increasingly moved away from this acronym in favor of the term “limited cutaneous systemic sclerosis” or just “limited scleroderma.” However, some physicians still use the term CREST Syndrome, as it can be easier for a patient to understand.

The terms limited, diffuse, and sine are diagnoses that are based on the extent of skin involvement seen on examination, but one is not considered more or less serious than the other. Persons with limited scleroderma generally have skin involvement that does not extend above the elbow or above the knee, although in some patients, the face can be affected. Those with diffuse scleroderma can have widespread skin thickening involving the entire body, and individuals with sine scleroderma will have nonexistent or extremely minor skin involvement. Sine scleroderma has all the internal organ manifestations of the disease, and this is generally the basis on which the diagnosis is made.

Always remember that this disease is highly individualized. Not every person who is diagnosed with systemic scleroderma will have the same presentation. Some may have a mild form of the disease while others may have more progressive disease. It is also important to understand that scleroderma is a life-long, chronic condition. There is no known cure, but there are therapies to address the symptoms of this disease. Natural progression of the disease is such that the first few years can be rocky with the onset of symptoms. Over time, progression of the disease will quiet down and some people will enjoy periods of remission.

There are several very important things that you and your physician can do to effectively manage your scleroderma. The most critical is early detection of problems to minimize or prevent complications. This is accomplished through a series of tests that are performed at the time of diagnosis to provide a baseline, and then conducted on a regular basis thereafter. It is imperative that every single person diagnosed with systemic scleroderma, whether limited or diffuse, has these done. These screenings can alert your physician to potential problem areas even before you become symptomatic. Early detection can make a difference in treatment options, and more importantly, on treatment outcomes.

Pulmonary arterial hypertension may occur in 25–50% of persons with limited scleroderma. Interstitial lung disease may occur in 10% of this population.

Renal involvement is less commonly associated with limited scleroderma, but is still a consideration. Patients should monitor their blood pressure several times per week to be alerted to any increases, as new onset hypertension can precipitate renal crisis.

Persons with the limited form of the disease generally have difficulty with their gastrointestinal tract. For a clear understanding of these complications, treatments and diagnostics, please refer to the following article: Medical Report: Digestive System (Gut, Gastro-intestinal) Involvement in Scleroderma.

One should also take great care to manage their Raynaud’s phenomenon. Minimizing attacks through practical lifestyle changes and protective measures can reduce the amount of damage from these vasospasms. If you would like more detailed information about this topic, we have several comprehensive articles that may be helpful. They are available on our website, www.scleroderma.org/medical/raynauds.shtm, or by calling our toll-free HelpLine at 800-722-HOPE (4673).

Equally important is scheduling regular visits with your rheumatologist. Your rheumatologist will be the quarterback of your health care team. He or she should coordinate the bulk of your care and monitor your serial diagnostics, referring you to other specialists as indicated. It is not unusual for some patients to consult with a pulmonologist, gastroenterologist, cardiologist, or nephrologist at various times in order to access the most appropriate treatment. Although juggling all these visits may seem overwhelming, doing things such as obtaining copies of your test results, keeping a record of your medications, and tracking the dates of your last office visits can help you to feel more in control of the situation. It is also important to make sure that each of these specialists communicates your test results and any medication changes or additions to your rheumatologist, so he or she has complete records. It is also a good idea to work with a physician with whom you can have an open and honest dialogue about new or changing symptoms, and who is willing to address any other reasonable concerns you may have.

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