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Results for “Scleroderma, Systemic”

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PubMed1979

Intestinal atony in progressive systemic sclerosis (Scleroderma).

A 27-year-old woman with a long history of scleroderma developed megacolon and severe fecal vomiting as a result of intestinal atony. Evacuation of fecal impaction with water-soluble contrast enema resulted in prompt relief of fecal vomiting and gradual return of bowel function.

Adult↗
PubMed1979

Pericardial tamponade in systemic sclerosis (scleroderma).

The frequency of pericardial disease in scleroderma found at necropsy in high. The clinical recognition of pericarditis with or without effusion is rare and tamponade with haemodynamic impairment is exceptional. Three patients with scleroderma presented with an acute syndrome of dyspnoea, chest pain, and cardiomegaly requiring pericardiocentesis for relief of pericardial tamponade. One patient died. The mechanism of the pericardial effusion remains unknown. The haemodynamic data recorded from one patient suggested that pericardial fibrosis in scleroderma may predispose to pericardial tamponade.

Adolescent↗
PubMed1989

Pulmonary hypertension in the CREST syndrome: variant of systemic sclerosis (scleroderma)--a case report.

The CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) is a variant but not a benign form of scleroderma. Pulmonary hypertension without pulmonary fibrosis appears to be more prevalent in the CREST than in scleroderma patients. Moreover, pulmonary hypertension in CREST may be rapidly progressive and a cause of sudden death from severe cor pulmonale.

Calcinosis↗
PubMed2004

[Systemic sclerosis (scleroderma) and malignancy].

The association of malignancy with collagen diseases has been a focus of interest. Especially, relation between dermatomyositis and malignancy is well-recognized. I reviewed here about the coexistence of SSc and malignancy in literature. I would like to raise four points as characteristics of the relationship. (1) Special attention should be paid to the patients who is late-onset, with advanced skin sclerosis (ex. Barnett III type) and male. (2) There are three generalized mechanisms of the association. The first is the predisposition of the malignancy on the basis of organ fibrosis, such as pulmonary fibrosis and lung cancer. The second is the nature of paraneoplastic syndrome. Lastly, immunological derangement inherent to SSc might cause carcinogenesis. (3) It has been documented that the interval of the onsets of SSc and breast cancer is extremely short. (4) Longstanding reflux esophagitis and Barrett's esophagus with SSc is suspected to predispose to esophageal carcinoma.

Female↗