Dermatology
Shibley Rahman, Avinash Sharma in A Complete MRCP(UK) Parts 1 and 2 Written Examination Revision Guide, 2018
CREST syndrome consists of: CalcinosisRaynaud’s phenomenon(o)Esophageal dysmotilitySclerodactylyTelangiectasia
Scleroderma (Systemic Sclerosis)
K. Gupta, P. Carmichael, A. Zumla in 100 Short Cases for the MRCP, 2020
Patients with the limited form of this disease, namely the CREST syndrome, have a relatively good prognosis. Prognosis is very dependent on the organ systems involved. Thus, renal involvement is associated with the worst prognosis, one estimate being a 30% 10-year survival while pulmonary involvement has a 50% 10-year survival. For patients with no cardiac, renal or pulmonary involvement, the 10-year survival has been estimated at 70%.
Hands
Tor Wo Chiu in Stone’s Plastic Surgery Facts, 2018
Systemic sclerosis/scleroderma is a rare multi-organ disease, affecting women three to six times more often. There is a polyarthritis in some patients (40% of whom are RF positive). Erosive arthritis is rare, but joint deformity may occur secondary to fibrous contracture. There are associations with the CREST syndrome, i.e. calcinosis circumscripta, digital ischaemia, etc.
Microscopic colitis and its associations with complications observed in classic inflammatory bowel disease: a systematic review
Published in Scandinavian Journal of Gastroenterology, 2020
Felix Solberg, Bodil Ohlsson
Rheumatic diseases were covered in four different articles [17,18,20,24]. Two out of four articles had separated their results into CC and LC [17,18]. Roth et al. [20] and Gu et al. [24] concluded that there was a significant association between MC and rheumatoid arthritis (RA) (OR: 7.21; 95% CI: 3.81–13.64 and p = .022, respectively). However, Wickbom et al. [17] showed a significant association between CC and rheumatic diseases (OR: 1.9; 95% CI: 1.0–3.5; p = .042), but the association between LC and rheumatic diseases was not statistically significant. Kao et al. [18] could however show a significant association between CC and RA and Reynaud/CREST syndrome (p < .01 for both). In addition, there was a significant association between LC and RA (p < .01), Reynaud/CREST syndrome (p < .01, fibromyalgia (p < .01), giant cell/temporal arteritis (p < .025) and systemic lupus erythematosus (SLE) (p < .025) [18].
Challenges and strategies for coping with scleroderma: implications for a scleroderma-specific self-management program
Published in Disability and Rehabilitation, 2019
Katherine Milette, Brett D. Thombs, Kristina Maiorino, Warren R. Nielson, Annett Körner, Sandra Peláez
According to the patients, since SSc is a rare, under-researched disease, it was a key challenge for them to find accurate information, either in the community or in the health care system, on how to manage the disease and deal with daily symptoms. Even patients who described themselves as being highly proactive faced difficulties coping. For instance, internet searches would often lead patients to inaccurate and misleading information. In this regard, Samantha (65 years old, CREST syndrome, employed part-time) shared the explicit warning she received from her treating physician: “My doctor was very careful to say: ‘Don’t go on the internet and read everything you see because a lot of it is going to be false information.’” Furthermore, the limited evidence-based information that was available was difficult to locate and would frequently not address all patient concerns, since SSc involves a large and varied spectrum of symptoms.
Oesophageal motor disorders and oesophageal endoscopic involvement in patients with systemic sclerosis: a systematic association?
Published in Scandinavian Journal of Gastroenterology, 2021
Véronique Vitton, Camille Bazin, Laure Luciano, Brigitte Granel, Marine Alessandrini, Jean-Robert Harle
Systemic sclerosis (SSc) is defined by excessive collagen deposition in the skin and other organs. It is a rare disease with an annual incidence of 130–160 persons/million inhabitants in France [1,2]. It predominantly affects women (female-to-male ratio of 3–8:1) with a frequency peak between 45 and 64 years old. Two main forms of SSc have been described: the diffuse cutaneous form (dSSc) and the limited cutaneous one (lSSc), defined according to the extent of cutaneous sclerosis [3]. There is also a particular form of lSSc known as CREST syndrome, defined by the presence of Calcinosis, Raynaud’s phenomenon, Oesophageal dysmotility, Sclerodactily and Telangiectasia [4].
Related Knowledge Centers
- Calcinosis
- Centromere
- Connective Tissue Disease
- Pulmonary Hypertension
- Sclerodactyly
- Systemic Scleroderma
- Telangiectasia
- Cell Nucleus
- Raynaud Syndrome
- Esophageal Motility Disorder