[Visceral manifestations of progressive scleroderma].
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In a 36-month prospective trial 21 patients with systemic sclerosis (diffuse systemic sclerosis 16 patients and 5 subjects with limited cutaneous subtype) were treated with D-penicillamine. In all patients with diffuse systemic sclerosis there was objective improvement. The degree and extent of skin involvement decreased significantly (p less than 0.001), whereas no objective improvement was noted in patients with limited cutaneous subtype. Further, no systemic progression of the disease was observed during the study period. Our results suggest that a prolonged treatment with D-penicillamine in small doses is not only beneficial and effective but also free of side-effects, if used at an earlier stage.
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In our study, the characteristics of 114 patients with systemic sclerosis (SSc) are discussed with emphasis on the subgroup of cases whose onset of disease occurred above the age of 60 years. Seven out of the 9 cases showed symptoms of diffuse cutaneous systemic sclerosis with an extensive skin involvement, and 5 of these cases died within 2 years following the onset of SSc. Seven of the 9 cases showed a rapid disease course with symptoms of cardiac, pulmonary and/or renal involvement, while no secondary Sjögren's syndrome, subcutaneous calcinosis and myositis were demonstrated among these patients.
This is a report of a patient with the CRST syndrome, a mild variant of scleroderma consisting of calcinosismraynaud's phenomenon, sclerodactyly, and telangiectasia. Typical changes of scleroderma were present in the extremities, esophagus, duodenum and colon. In addition, there was a polypoid filling defect in a colonic diverticulum due to a fecalith. The radiologic appearance at first resembled a colonic neoplasm, although its location within a diverticulum and its speckled appearance suggested the possibility of a fecalith. This was confirmed at colonoscopy, which disclosed numerous wide-mouthed diverticula, with inspissated fecal material projecting from several diverticula. In patients with scleroderma and polypoid filling defects in the colon, the possibility of a fecalith within a diverticulum should be considered. Where the radiologic study is inconclusive, colonoscopy may provide a definitive diagnosis.
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The recent description of a large cohort of patients with the diagnosis of systemic sclerosis sine scleroderma (ssSSc) provided significant progress in our understanding of this entity. The prognosis of patients with ssSSc is, however, very variable, from benign in most cases to rapidly disabling in others. By reporting three new cases and analyzing previously published data, we discuss possible subsets and variants of the disease form.
Fibrosis is characterized by the increased deposition of collagen and other matrix components by fibroblasts. This process occurs as a reaction to inflammation and is mediated by numerous cytokines including transforming growth factor beta (TGF-beta). Localized cutaneous scleroderma or morphea is characterized by fibrosis. Current treatment for morphea includes topical, intralesional, or systemic corticosteroids, vitamin D analog (calcitriol and calcipotriol), photochemotherapy, laser therapy, antimalarials, phenytoin, D-penicillamine, and colchicine, all with varying degrees of success. In this case report, imiquimod cream 5% (Aldara), which induces interferon and in turn inhibits TGF-beta, was employed to treat morphea.
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The CRST syndrome is defined, and its relation to systemic sclerosis described. The literature referring to the condition is reviewed and a further case reported. Clinical and diagnostic features of the telangiectasia are emphasised, particularly in comparison with those of the similar condition, hereditary haemorrhagic telangiectasia.
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