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Scleroderma (Systemic Sclerosis)
Published in K. Gupta, P. Carmichael, A. Zumla, 100 Short Cases for the MRCP, 2020
K. Gupta, P. Carmichael, A. Zumla
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
Published in Tor Wo Chiu, 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.
Dermatology
Published in Shibley Rahman, Avinash Sharma, A Complete MRCP(UK) Parts 1 and 2 Written Examination Revision Guide, 2018
Shibley Rahman, Avinash Sharma
CREST syndrome consists of: CalcinosisRaynaud’s phenomenon(o)Esophageal dysmotilitySclerodactylyTelangiectasia
Scleroderma patient perspectives on social support from close social relationships
Published in Disability and Rehabilitation, 2020
Katherine Milette, Brett D. Thombs, Sébastien Dewez, Annett Körner, Sandra Peláez
Although 25 patients originally signed up for the focus groups, a total of 19 patients with SSc participated in the focus groups (6 drop-outs: 1 = sudden onset of gastrointestinal symptoms, 1 = focus group conflicted with conference schedule, 4 = unknown). Seventeen patients were women (1st focus group =4 females, 2nd = 4 females, 3rd = 4 females, 4th = 5 females, 2 males) and the age of patients ranged from 31 to 74 years old with a mean age of 57.1 years old (SD =10.9). The mean number of years since receiving a SSc diagnosis was 18.2 years (SD =13.2). While some patients had received their diagnosis only a year prior, others had been living with the diagnosis for up to 46 years. Seven (37%) patients reported a diagnosis of diffuse SSc; three (16%) reported limited SSc; nine (47%) reported CREST syndrome, which is a term used in the past to denote limited SSc and an acronym that stands for the main features of the disease, which are Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia. A total of 12 (63%) patients indicated being White and 15 (79%) had completed at least some college/university education. A more detailed summary of patient characteristics is provided in Table 2.
Microscopic colitis and its associations with complications observed in classic inflammatory bowel disease: a systematic review
Published in Scandinavian Journal of Gastroenterology, 2020
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
Some SSc patients are not followed by health care professionals who are knowledgeable or specialize in SSc. They mentioned that because their health care professionals were not sufficiently knowledgeable about the disease, they felt they were not being properly followed in terms of their medical care. Lack of disease-related knowledge by the SSc patients themselves further contributed to problems during medical visits, including not being well prepared to ask for more information and not knowing how to speak up about their needs. Jamie (71 years old, CREST syndrome, retired) explained her experience: “When I was first diagnosed, I didn’t have [any of the symptoms] yet and so I didn’t know what to ask [my doctors] because I didn’t know what I was going to get.”