Documenti di Didattica
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Lauren T. Kalanta
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
(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
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
CREST SYNDROME 3
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
CREST syndrome (Lewis, Dirkesen, & Heithkemper, 2013). Mortality rates in patients with
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
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, &
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
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
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,
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,
CREST SYNDROME 7
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