lcSSc

limited cutaneous systemic sclerosis

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/03/14 17:07:09」(JST)

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CREST syndrome
Classification and external resources
ICD-10	M34.1
ICD-9	710.1
OMIM	181750
DiseasesDB	29764
MeSH	D017675

CREST syndrome, also known as the limited cutaneous form of systemic sclerosis (lcSSc) is a multisystem connective tissue disorder. The initialism "CREST" refers to the five main features: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia. It is associated with detectable antibodies against centromeres (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

Calcinosis

CREST/lcSSc causes thickening and tightening of the skin with deposition of calcific nodules ("calcinosis").

Raynaud's phenomenon

Raynaud's phenomenon is frequently the first manifestation of CREST/lcSSc, preceding other symptoms by years. Stress and cold temperature induces 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 transitions in skin color. Underlying this transition is pallor and cyanosis of the digits, followed by a reactive hyperemia as they rewarm. In contrast to uncomplicated primary Raynaud phenomenon, this occurrence is often painful. Frequently this phenomenon leads to digital ulcerations, gangrene, or amputation. Ulceration can predispose to chronic infections of the involved site.

Esophageal dysmotility

Presents as a sensation of food getting stuck (dysphagia) in the mid or lower esophagus, atypical chest pain, or cough. Patients often state they must drink liquids to swallow solid food. This motiltiy problem results from atrophy of the gastrointestinal tract wall smooth muscle. This change may occur with or without pathologic evidence of significant tissue fibrosis.

Sclerodactyly

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 inflammed. Eventually, dermal fibroblasts overproduce extracellular matrix leading to increased tissue collage 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.

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 those patients with other scleroderma related vascular disease (ie, 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, 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).

Etiology

Crest syndrome involves the production of autoimmune anti-nuclear and anti-centromere antibodies, though their etiology is not currently understood. There is no known infectious cause.

Treatment

Disease progression may be slowed with 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.

Epidemiology

CREST syndrome can be noted in up to 10% of patients with primary biliary cirrhosis.

History

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.

See also

• Scleroderma

References

UpToDate Contents

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• 1. 強皮症の概要および分類 overview and classification of scleroderma disorders
• 2. 成人における全身性硬化症（強皮症）の治療および予後の概要 overview of the treatment and prognosis of systemic sclerosis scleroderma in adults
• 3. 成人における全身性硬化症（強皮症）の臨床症状の概要 overview of the clinical manifestations of systemic sclerosis scleroderma in adults
• 4. 成人における全身性硬化症（強皮症）の診断および鑑別診断 diagnosis and differential diagnosis of systemic sclerosis scleroderma in adults
• 5. 全身性硬化症（強皮症）性肺疾患の臨床症状 clinical manifestations of systemic sclerosis scleroderma lung disease

English Journal

• Detection of hair-microRNAs as the novel potent biomarker: Evaluation of the usefulness for the diagnosis of scleroderma.

• Wang Z, Jinnin M, Kudo H, Inoue K, Nakayama W, Honda N, Makino K, Kajihara I, Fukushima S, Inoue Y, Ihn H.SourceDepartment of Dermatology and Plastic Surgery, Faculty of Life Sciences, Kumamoto University, Honjo 1-1-1, Kumamoto 860-8556, Japan.
• Journal of dermatological science.J Dermatol Sci.2013 Nov;72(2):134-41. doi: 10.1016/j.jdermsci.2013.06.018. Epub 2013 Jul 10.
• BACKGROUND: Serum microRNA levels are known as useful biomarkers for various diseases. Recent publication has indicated the existence of microRNAs in hair roots and hair shafts.OBJECTIVE: In this study, we evaluated several methods for the extraction of hair microRNAs, and their usefulness for the d
• PMID 23890704

• Increased expression of S100A8 and S100A9 in patients with diffuse cutaneous systemic sclerosis. A correlation with organ involvement and immunological abnormalities.

• Xu X, Wu WY, Tu WZ, Chu HY, Zhu XX, Liang MR, Xue Y, Wang JC, Zou HJ.SourceDivision of Rheumatology, Huashan Hospital, Fudan University, No. 12, Middle Wulumuqi Road, Shanghai, 200040, People's Republic of China.
• Clinical rheumatology.Clin Rheumatol.2013 Oct;32(10):1501-10. doi: 10.1007/s10067-013-2305-4. Epub 2013 Jun 11.
• S100A8 and S100A9 play important roles in immune and inflammatory disorders. The role of the two proteins in systemic sclerosis (SSc) remains unknown. Fifty-seven diffuse cutaneous SSc (dcSSc) patients, 31 limited cutaneous SSc (lcSSc) patients were recruited in the present study. The expression of
• PMID 23754244

• Circulating microparticles and plasma levels of soluble E- and P-selectins in patients with systemic sclerosis.

• Iversen L, Ostergaard O, Ullman S, Nielsen C, Halberg P, Karlsmark T, Heegaard N, Jacobsen S.SourceDepartment of Dermatology, Bispebjerg Hospital, Copenhagen University Hospital, Copenhagen , Denmark.
• Scandinavian journal of rheumatology.Scand J Rheumatol.2013 Sep 9. [Epub ahead of print]
• Objectives: Microparticles (MPs) may be involved in the pathogenesis of systemic sclerosis (SSc), which includes vasculopathy, endothelial cell activation, and coagulation activation. Circulating MPs from SSc patients were characterized and their relationship with soluble markers of vascular activat
• PMID 24016306

Japanese Journal

• Acute Right Heart Failure and Achalasia-like Syndrome in a Patient with Limited Cutaneous Systemic Sclerosis and Primary Biliary Cirrhosis

• Nomoto Takahiko,Kikuchi Takanori,Kusunoki Yukiko,Akesaka Kazuyuki,Yano Kazuo,Fujii Yasuhisa,Makino Hideichi
• Internal Medicine 49(16), 1779-1782, 2010
• … We report a case of a 63-year-old woman who developed acute right heart failure and an achalasia-like syndrome with limited cutaneous systemic sclerosis (lcSSc) and primary biliary cirrhosis. … Though rare, physicians should be aware that some patients with lcSSc may develop acute right heart failure or achalasia-like syndrome. …
• NAID 130000299642

• Association of IL-10 receptor 2 (IL10RB) SNP with systemic sclerosis

• Hikami Koki,Ehara Yukikazu,Hasegawa Minoru,Fujimoto Manabu,Matsushita Masaki,Oka Takanori,Takehara Kazuhiko,Sato Shinichi,Tokunaga Katsushi,Tsuchiya Naoyuki,土屋 尚之
• Biochemical and biophysical research communications 373(3), 403-407, 2008-08
• … Significant association was not observed in limited cutaneous SSc (lcSSc). …
• NAID 120000830198

Related Links

• Scleroderma - Limited Cutaneous Systemic Sclerosis

Pulmonary hypertension is recognised as a lethal complication of all forms of systemic sclerosis but especially in the limited form of the condition. To download further information on Limited Cutaneous Systemic Sclerosis (lcSSc) click here

• Disorders of the scleroderma family: the importance of the ... - eustar

systemic sclerosis (lcSSc). -diffus cutaneous systemic sclerosis (dcSSc). - scleroderma sine scleroderma. Skin involvement of the acral regions. Skin involvement of the trunk and upper part of extremities. No skin scleroderma, only internal ...

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