Running head: CREST SYNDROME 1

Scleroderma: CREST Syndrome

Lauren T. Kalanta

California State University, Stanislaus

Scleroderma: CREST Syndrome

CREST SYNDROME 2

Evidence-based teaching is an invaluable and highly used fundamental of nursing.

Scleroderma, otherwise known as systemic sclerosis, is a connective tissue disorder that is marked by

degenerative, fibrogenic, and inflammatory changes in multiple body systems such as the

integumentary system, cardiovascular system, pulmonary system, and musculoskeletal system

(Poole, MacIntyre, & DeBoer, 2013). Scleroderma is an autoimmune, non-hereditary disease that

affects 3 in 10,000 people (Lewis, Dirkesen, & Heithkemper, 2013). Women have a greater

incidence of having scleroderma than males and are usually diagnosed starting around age 30

(Poole, MacIntyre, & DeBoer, 2013). Diffuse cutaneous scleroderma and limited cutaneous

scleroderma are the two types of scleroderma with limited cutaneous scleroderma being more

common and diffuse scleroderma having a less favorable prognosis (Poole, MacIntyre, &

DeBoer, 2013). CREST syndrome is a subgroup of scleroderma that is comprised of five distinct

features (Poole, MacIntyre, & DeBoer, 2013). CREST is an acronym for the manifestations of

the syndrome; its features include calcinosis, Raynaud's phenomenon, esophageal abnormalities,

sclerodactyly, and telangiectasia (Lewis, Dirkesen, & Heithkemper, 2013). Since having some or

all of these features poses risk to multiple organ systems, patients with CREST syndrome should

be monitored and educated about potential risks and evidence-based management and treatments

(Poole, MacIntyre, & DeBoer, 2013).

Pathophysiology

Calcinosis, which is a buildup of calcium in the skin, is the first letter in the CREST

acronym and is very painful for the patient (Lewis, Dirkesen, & Heithkemper, 2013). The

deposits can cause hard and thick patches that obstruct circulation to the skin that can eventually

lead to amputation (Lewis, Dirkesen, & Heithkemper, 2013). Raynauds phenomenon, the

second letter in the acronym CREST, is common and occurs in 86% to 98% of people with

CREST syndrome (Poole, MacIntyre, & DeBoer, 2013). This phenomenon may cause pain and
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can contribute to skin ulcers because it comprises of abnormal blood flow to the tissues of the

hands and feet (Poole, MacIntyre, & DeBoer, 2013). It is typically the first symptom seen with

limited scleroderma (Lewis, Dirkesen, & Heithkemper, 2013). The patient may complain of

tingling or numbness after experiencing cyanosis and then erythema stemming from a Raynauds

episode (Lewis, Dirkesen, & Heithkemper, 2013). The third letter in CREST syndrome stands for

esophageal abnormalities (Poole, MacIntyre, & DeBoer, 2013). Changes in organ systems such

as the gastrointestinal tract, lungs, heart, and kidneys occur early on in diffuse systemic sclerosis

(Poole, MacIntyre, & DeBoer, 2013). Gastrointestinal upset and heartburn are characteristics of

the esophageal aspect of CREST syndrome and typically involves 66% to 90% of people with

the syndrome (Poole, MacIntyre, & DeBoer, 2013). Sclerodactyly, which stands for the fourth

letter in CREST, is the tightening of skin around the joints of the digits on both the hands and the

feet (Poole, MacIntyre, & DeBoer, 2013). Restricted range of motion (ROM) in the hands and

face are findings with sclerodactyly (Poole, MacIntyre, & DeBoer, 2013). With limited cutaneous

scleroderma, thickening and swelling is usually localized to forearms and shins and does not

extend past the elbow or knee (Lewis, Dirkesen, & Heithkemper, 2013). Telangiectasias, the fifth

letter in CREST, are red spots found all over the body but not limited to hands, forearms, lips,

and face (Lewis, Dirkesen, & Heithkemper, 2013). If a patient is found to have some or all of

these five characteristics, the diagnosis of CREST syndrome can be made (Lewis, Dirkesen, &

Heithkemper, 2013).

Potential Risks

Unfortunately, there are no current methods to prevent scleroderma and, therefore,

CREST syndrome (Lewis, Dirkesen, & Heithkemper, 2013). Mortality rates in patients with

diffuse cutaneous scleroderma result from cardiovascular, pulmonary, and gastrointestinal

CREST SYNDROME 4

complications (Poole, MacIntyre, & DeBoer, 2013). From the time of diagnosis, mortality rates

have found to occur within 5 years of 77.9% of patients, within 10 years of 55.1% of patients,

and within 20 years of 26.8% of patients (Poole, MacIntyre, & DeBoer, 2013). Predicting the

survival rates in patients with diffuse cutaneous scleroderma depends on the amount of skin

involved (Poole, MacIntyre, & DeBoer, 2013). It is predicted that patients with only

sclerodactyly have higher survival rates than those without because renal complications are

found to be less frequent (Poole, MacIntyre, & DeBoer, 2013). The involvement of the renal

system is the largest contributing factor for scleroderma related deaths (Poole, MacIntyre, &

DeBoer, 2013). When renal insufficiency is suspected, there may be protein and blood found in

the urine (Lewis, Dirkesen, & Heithkemper, 2013). Patients with CREST syndrome often

develop dysphagia, dental caries, gum disease, constipation, diarrhea, dry mouth, and dry eyes

which often leads to decreased food intake and, eventually, weight loss (Lewis, Dirkesen, &

Heithkemper, 2013). The pulmonary system becomes affected when the pleural lining of the

lungs becomes thickened and functional abnormalities occur (Lewis, Dirkesen, & Heithkemper,

2013). These abnormalities can lead to pulmonary arterial hypertension and lung disease (Lewis,

Dirkesen, & Heithkemper, 2013). Because of CREST syndromes ability to affect multiple

systems, diagnosed patients should be carefully monitored (Lewis, Dirkesen, & Heithkemper,

2013).

Collaborative Care

In the collaborative care of scleroderma, the main focus is supportive care geared towards

treating secondary damage to organ systems and preventing further damage from occurring

(Lewis, Dirkesen, & Heithkemper, 2013). The collaborative team should be comprised of

occupational therapists in order to maintain activities of daily living, physical therapists to

CREST SYNDROME 5

maintain joint mobility and muscle strength, and several other members of the healthcare team

(Lewis, Dirkesen, & Heithkemper, 2013). Team members within the hospital setting, such as

technicians and pharmacists, need to collaborate together to perform diagnostic testing including

urinalysis, skin biopsies, chest x-rays, ECG, and pulmonary function tests (Lewis, Dirkesen, &

Heithkemper, 2013). Team members such as physicians and nurses, must work together to

perform assessments, define a detailed , medical history, and administer medications; however,

medication has not been found to be an effective treatment for scleroderma (Lewis, Dirkesen, &

Heithkemper, 2013). Drug combinations such as beta-blockers, vasoactive agents, vasodilators,

and alpha-adrenergic blocking agents are used to treat the characteristics of CREST syndrome

such as Raynauds phenomenon (Lewis, Dirkesen, & Heithkemper, 2013). The collaborative

efforts of healthcare providers will ensure that sclerodermas resulting in CREST syndrome will

be maintained and managed (Lewis, Dirkesen, & Heithkemper, 2013).

Evidence-Based Nursing Management

The main theme surrounding the management and treatment of CREST syndrome is

comprised of education about all therapies and medications, thorough explanation of all

procedures and treatments, and stressing the importance of seeking regular medical care (Lewis,

Dirkesen, & Heithkemper, 2013). All of these aspects will help to improve quality of life and

performance of activities of daily living (Poole, MacIntyre, & DeBoer, 2013). Surprisingly,

laboratory finding for patients with scleroderma are found to be within defined limits (Lewis,

Dirkesen, & Heithkemper, 2013). Detection begins when a patient begins to show symptoms

such as Raynaud's phenomenon or sclerodactyly (Lewis, Dirkesen, & Heithkemper, 2013).

Nursing interventions within the hospital begin with taking vital signs, assessing respiratory

function, taking daily weight, measuring intake and output, and assessing bowel function
CREST SYNDROME 6

(Lewis, Dirkesen, & Heithkemper, 2013). Patients should be instructed to not perform finger-

stick blood sugar testing because of decreased wound healing stemming from poor circulation of

the hands (Lewis, Dirkesen, & Heithkemper, 2013). A diagnosis of systemic sclerosis can be

devastating to a patient and nurses should be available to provide emotional support and

education (Lewis, Dirkesen, & Heithkemper, 2013). In order to avoid fatigue, healthcare

providers should follow the four P's of energy conservation which include prioritization,

planning, pacing and posture (Poole, MacIntyre, & DeBoer, 2013). Following a carefully

organized plan of care that follows the patients rate of progression is the best option (Poole,

MacIntyre, & DeBoer, 2013). Restoring and maintaining a wide range of ROM activities is

essential for the restoration activities of daily living (Poole, MacIntyre, & DeBoer, 2013). In

order to reduce pain and increase collagen tissue resilience before performing ROM exercises,

applying heat using paraffin wax or hot packs should be utilized (Poole, MacIntyre, & DeBoer,

2013). Physiotherapy has been found to minimize hand and mouth disabilities involved with

scleroderma (Poole, MacIntyre, & DeBoer, 2013). Patients should also be taught to protect hands

and feet from the cold, to apply lotion to dry areas, and to use assistive devices when necessary

(Poole, MacIntyre, & DeBoer, 2013). All of these methods have been proven to manage CREST

syndrome and should be continued until further advancements are discovered (Poole, MacIntyre,

& DeBoer, 2013).

Despite scleroderma and resulting CREST syndrome having no definitive cure, the

patients quality of life can still be preserved through collaborative efforts of the healthcare team

(Lewis, Dirkesen, & Heithkemper, 2013). Recognizing early on the characteristics of the

syndrome and intervening with diagnostics and laboratory findings can prevent further organ

damage and preserve the patients existing intact systems (Lewis, Dirkesen, & Heithkemper,
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2013). The effects of CREST syndrome can be dire, but with the intervention of a collaborative,

skilled healthcare team, the outcome for the patient looks much brighter.

References

Lewis, S.M., Dirkesen, S.R., Heithkemper, M.M., Bucher, S., (2013). Medical surgical

nursing: Assessment and management of clinical problems. (8th ed.). St Louis, MO:

Mosby.

Poole, L., MacIntyre, N., DeBoer, H. (2013). Evidence based management of hand and

mouth disability in a woman living with diffuse systemic sclerosis (scleroderma).

Physiotherapy Canada, 65(4), 317-320.