CREST syndrome

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Classification according to ICD-10
M34.1 CR (E) ST syndrome
ICD-10 online (WHO version 2019)

The CREST syndrome is a special form of systemic sclerosis . The acronym stands for the main features of C alcinose , R Aynaud syndrome , impaired peristalsis in the esophagus ( E esophagus), S klerodaktylie and T eleangiektasie .

The similar Thibièrge-Weissenbach syndrome has a less favorable course.

Synonyms are: calcinosis - Raynaud's phenomenon - esophageal dysfunction - sclerodactyly - telangiectasia; CR (E) ST syndrome; CRST syndrome; English Scleroderma, familial progressive; Susceptibility to Systemic Sclerosis

The first description comes from the year 1964 by RH Winterbauer as CRST syndrome.

The name "CREST" was proposed in 1973 by RA Frayha and colleagues.

distribution

The frequency is given as about 1 in 12,500, women are affected about 4 times more often.

root cause

The cause is not known, it starts in an immune response with overproduction of collagen .

Clinical manifestations

Clinical criteria are:

  • Manifestation adulthood usually between 40 and 50 years
  • Raynaud's syndrome, the most common and often the first clue
  • Skin changes limited to fingers and face
  • Calcinosis on the limbs around the joints
  • Sclerodactyly starting at the fingertips
  • Telangiectasias on the hands, face and lips
  • gastroesophageal reflux of the esophagus , possibly also dysphagia

diagnosis

The diagnosis is based on the clinical findings and the course. Capillaroscopy reveals typical microangiopathies with megacapillaries.

In blood serum typical to anti-centromere autoantibodies (ACA) are detected.

Differential diagnosis

The following are to be distinguished:

therapy

A causal treatment is not known. Various symptoms can be treated accordingly.

course

The disease progresses slowly; pulmonary hypertension (30–40%) as a complication or pulmonary fibrosis (10%) can develop.

literature

  • M. Tajalli, AA Qureshi: Successful treatment of calcinosis cutis of fingertip in the setting of CREST syndrome with topical 20% sodium thiosulfate. In: JAAD case reports. Volume 5, number 11, November 2019, pp. 988-990, doi: 10.1016 / j.jdcr.2019.08.011 , PMID 31709293 , PMC 6835016 (free full text).
  • S. Fuente Cosío, B. Arca Barca, P. Martínez García, A. Sampedro López: Chronic Anterior Uveitis in a Patient with CREST Syndrome. In: Reumatologia clinica. [electronic publication before printing] January 2019, doi: 10.1016 / j.reuma.2018.10.005 , PMID 30691950 .

Individual evidence

  1. ↑ a b c d e f g h CREST syndrome. In: Orphanet (Rare Disease Database).
  2. ↑ Encyclopedia Dermatology
  3. ↑ emedicine
  4. ^ Theuma-online
  5. ^ RH Winterbauer: Multiple telangiectasia, Raynaud's phenomenon, sclerodactyly, and subcutanious calcinosis: a syndrome mimicking hereditary hemorrhagic telangiectasia. In: Bulletin of the Johns Hopkins Hospital. Volume 114, June 1964, pp. 361-383, PMID 14171636 .
  6. ↑ RA Frayha, YES Scarola, LE Shulman: calcinosis in scleroderma: A reevaluation of the CRST syndrome, abstracted. In: Arthritis & Rheumatology Vol. 16, 1973, pp. 16: 542.
  7. ↑ R. Gläser, A. Weidinger: New classification criteria for systemic scleroderma taking into account capillary microscopy. In: The dermatologist; Journal of Dermatology, Venereology, and Allied Fields. Volume 66, Number 6, June 2015, pp. 487-488, doi: 10.1007 / s00105-015-3639-4 , PMID 25952023 .

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