CREST syndrome: Difference between revisions

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{{Infobox medical condition
| name = CREST syndrome
| image = Scleroderma.jpg
| caption = Skin changes in scleroderma
| field = Rheumatology
| synonyms = Limited scleroderma
| symptoms = [[Calcinosis]], [[Raynaud's phenomenon]], [[Esophageal dysmotility]], [[Sclerodactyly]], [[Telangiectasia]]
| complications = [[Pulmonary hypertension]], [[Gastroesophageal reflux disease]]
| onset = Middle age
| duration = Long term
| causes = [[Autoimmune disease]]
| risks = [[Family history]], [[Environmental factors]]
| diagnosis = [[Clinical evaluation]], [[Antibody testing]]
| treatment = [[Medications]], [[Physical therapy]], [[Lifestyle changes]]
| prognosis = Variable
| frequency = Rare
}}


'''CREST syndrome''', also known as the '''limited cutaneous''' form of '''[[systemic sclerosis]]''' (lcSSc), is a multisystem [[connective tissue disease|connective tissue disorder]]. The acronym "CREST" refers to the five main features: [[calcinosis]], [[Raynaud's phenomenon]], [[Esophageal motility disorder|esophageal dysmotility]], [[sclerodactyly]], and [[telangiectasia]].
'''CREST syndrome''' is a subtype of [[systemic sclerosis]], also known as limited scleroderma. It is characterized by a specific set of symptoms that form the acronym CREST: [[Calcinosis]], [[Raynaud's phenomenon]], [[Esophageal dysmotility]], [[Sclerodactyly]], and [[Telangiectasia]].


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==Signs and Symptoms==
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CREST syndrome is associated with detectable antibodies against [[centromere]]s (a component of the [[cell nucleus]]), and usually spares the kidneys (a feature more common in the related condition [[systemic scleroderma]]). If the lungs are involved, it is usually in the form of [[pulmonary arterial hypertension]].
==Signs and symptoms==
[[File:CREST2015.JPG|thumb|CREST syndrome (calcinosis and sclerodactyly)]]
[[File:Calcinosis of CREST syndrome.jpg|thumb|[[Projectional radiography|X-rays]] showing calcinosis in a woman with CREST syndrome.]]
[[File:CRESTsyndrome.png|thumb|X-ray of subtle calcifications in CREST syndrome.]]
===Calcinosis===
===Calcinosis===
CREST causes thickening and tightening of the skin with deposition of calcific nodules ("calcinosis").
Calcinosis refers to the deposition of calcium salts in the skin and tissues. It can lead to painful nodules and skin ulcerations, particularly on the fingers and other pressure points.
 
===Raynaud's phenomenon===
 
[[Raynaud's phenomenon]] is frequently the first manifestation of CREST/lcSSc, preceding other symptoms by years. Stress and cold temperature induce an exaggerated vasoconstriction of the small arteries, arterioles, and thermoregulatory vessels of the skin of the digits. Clinically this manifests as a white-blue-red transition in skin color.  Underlying this transition is pallor and cyanosis of the digits, followed by a reactive hyperemia as they rewarm.


When extreme and frequent, this phenomenon can lead to digital ulcerations, gangrene, or amputation.
===Raynaud's Phenomenon===
Ulceration can predispose to chronic infections of the involved site.
Raynaud's phenomenon is a condition where the blood vessels in the fingers and toes constrict excessively in response to cold or stress, leading to color changes, pain, and sometimes ulceration.


===Esophageal dysmotility===
===Esophageal Dysmotility===
Presents as a sensation of food getting stuck ([[dysphagia]]) in the mid- or lower esophagus, atypical chest pain, or cough. People often state they must drink liquids to swallow solid food.  This motility problem results from atrophy of the gastrointestinal tract wall smooth muscle.  This change may occur with or without pathologic evidence of significant tissue fibrosis.
Esophageal dysmotility involves the abnormal movement of the esophagus, which can cause difficulty swallowing, [[gastroesophageal reflux disease]] (GERD), and other digestive issues.


===Sclerodactyly===
===Sclerodactyly===
Sclerodactyly is the thickening and tightening of the skin on the fingers and toes. This can lead to decreased mobility and function of the digits.


Though it is the most easily recognizable manifestation, it is not prominent in all patients. Thickening generally only involves the skin of the fingers distal to the metacarpophalangeal joints in CREST.  Early in the course of the disease, the skin may appear edematous and inflamed.  Eventually, dermal fibroblasts overproduce extracellular matrix leading to increased tissue collagen deposition in the skin.  Collagen cross-linking then causes a progressive skin tightening.  Digital ischemic ulcers commonly form on the distal fingers in 30-50% of patients.
===Telangiectasia===
 
Telangiectasia are small, dilated blood vessels near the surface of the skin or mucous membranes, appearing as red spots or lines.
===Telangiectasias===
Marked [[telangiectasias]] (dilated capillaries) occur on the skin of the face, the palmar surface of the hands, and the mucous membranes. Telangiectasias tend to be more numerous in people with other scleroderma related vascular disease (i.e., pulmonary arterial hypertension). The number of telangiectasias and the sites involved tend to increase over time.
 
===Other===
Other symptoms of CREST syndrome can be exhaustion, weakness, difficulties with breathing, pain in hands and feet, dizziness and badly healing wounds.
 
Patients with lcSSc commonly induce pulmonary artery hypertension which may result in [[cor pulmonale]] ([[heart failure]] due to increased pulmonary artery pressure).


==Cause==
==Causes==
Crest syndrome involves the production of autoimmune [[anti-nuclear antibodies|anti-nuclear]] and [[anti-centromere antibodies]], though their cause is not currently understood.  There is no known infectious cause.
CREST syndrome is an [[autoimmune disease]], meaning the body's immune system mistakenly attacks its own tissues. The exact cause is unknown, but it is believed to involve a combination of genetic and environmental factors.


==Diagnosis==
==Diagnosis==
CREST is not easily diagnosed as it closely mimics symptoms of other connective tissue and autoimmune diseases. Diagnoses are usually given when a patient presents two or more of the five major clinical symptoms.
Diagnosis of CREST syndrome is based on clinical evaluation and the presence of specific [[antibodies]] in the blood, such as [[anticentromere antibodies]].


==Treatment==
==Treatment==
Disease progression may be slowed with [[Immunosuppressive drug|immunosuppressives]] and other medications, and esophageal reflux, [[pulmonary hypertension]] and Raynaud phenomenon may benefit from symptomatic treatment. However, there is no cure for this disease as there is no cure for scleroderma in general.
Treatment for CREST syndrome focuses on managing symptoms and preventing complications. This may include:
* [[Medications]] such as [[calcium channel blockers]] for Raynaud's phenomenon and [[proton pump inhibitors]] for esophageal symptoms.
* [[Physical therapy]] to maintain mobility and function.
* [[Lifestyle changes]] such as avoiding cold exposure and smoking cessation.


==Epidemiology==
==Prognosis==
CREST syndrome can be noted in up to 10% of patients with [[primary biliary cirrhosis]].
The prognosis for individuals with CREST syndrome varies. Some may experience mild symptoms, while others may develop serious complications such as [[pulmonary hypertension]].


===History===
==See Also==
The combination of symptoms was first reported in 1964 by R.H. Winterbauer, at that point a medical student at [[Johns Hopkins School of Medicine]].<ref name=Winterbauer/>
* [[Systemic sclerosis]]
* [[Autoimmune disease]]
* [[Raynaud's phenomenon]]


==See also==
==External Links==
*[[Scleroderma]]
* [https://www.scleroderma.org/ Scleroderma Foundation]


==References==
{{Medical conditions related to autoimmunity}}
{{reflist}}
{{Systemic connective tissue disorders}}


{{Authority control}}
[[Category:Connective tissue diseases]]
[[Category:Systemic connective tissue disorders]]
[[Category:Autoimmune diseases]]
[[Category:Autoimmune diseases]]
[[Category:Syndromes]]
[[Category:Rheumatology]]
[[Category:Rare diseases]]

Revision as of 12:34, 31 December 2024

CREST syndrome
Scleroderma.jpg
Synonyms Limited scleroderma
Pronounce N/A
Specialty N/A
Symptoms Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia
Complications Pulmonary hypertension, Gastroesophageal reflux disease
Onset Middle age
Duration Long term
Types N/A
Causes Autoimmune disease
Risks Family history, Environmental factors
Diagnosis Clinical evaluation, Antibody testing
Differential diagnosis N/A
Prevention N/A
Treatment Medications, Physical therapy, Lifestyle changes
Medication N/A
Prognosis Variable
Frequency Rare
Deaths N/A


CREST syndrome is a subtype of systemic sclerosis, also known as limited scleroderma. It is characterized by a specific set of symptoms that form the acronym CREST: Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia.

Signs and Symptoms

Calcinosis

Calcinosis refers to the deposition of calcium salts in the skin and tissues. It can lead to painful nodules and skin ulcerations, particularly on the fingers and other pressure points.

Raynaud's Phenomenon

Raynaud's phenomenon is a condition where the blood vessels in the fingers and toes constrict excessively in response to cold or stress, leading to color changes, pain, and sometimes ulceration.

Esophageal Dysmotility

Esophageal dysmotility involves the abnormal movement of the esophagus, which can cause difficulty swallowing, gastroesophageal reflux disease (GERD), and other digestive issues.

Sclerodactyly

Sclerodactyly is the thickening and tightening of the skin on the fingers and toes. This can lead to decreased mobility and function of the digits.

Telangiectasia

Telangiectasia are small, dilated blood vessels near the surface of the skin or mucous membranes, appearing as red spots or lines.

Causes

CREST syndrome is an autoimmune disease, meaning the body's immune system mistakenly attacks its own tissues. The exact cause is unknown, but it is believed to involve a combination of genetic and environmental factors.

Diagnosis

Diagnosis of CREST syndrome is based on clinical evaluation and the presence of specific antibodies in the blood, such as anticentromere antibodies.

Treatment

Treatment for CREST syndrome focuses on managing symptoms and preventing complications. This may include:

Prognosis

The prognosis for individuals with CREST syndrome varies. Some may experience mild symptoms, while others may develop serious complications such as pulmonary hypertension.

See Also

External Links

Template:Medical conditions related to autoimmunity

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