PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “Scleroderma, Systemic”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 253 records · Page 14Linked to original sources
PubMed1988

[Treatment of systemic scleroderma using plasma exchange. A study of 19 cases].

Generalized scleroderma (GS) is associated with dysimmunity anomalies suggesting possible benefits of plasma exchange (PE) therapy. Nineteen patients with GS were treated by PE (volume of plasma exchange equivalent to 5-6% body weight and replacement by 4% human albumin), initially three times weekly, then weekly, bi-monthly and monthly (total duration 12-18 months). Clinical and paraclinical follow up was for an average of more than 2 years after the end of PE (mean number 17 per patient). Clinical results were assessed as positive and lasting in 11 cases (57.9%), two cases remaining stable and three cases worsening (one death from heart failure). The remaining three cases were failures in application of treatment (difficult venous approach). Improvement was noted in cutaneous sclerosis (62% of cases), trophic disorders (recovery in 6 of 7 cases) and articular manifestations. Vasomotor disorders were improved in only 20% of cases and visceral lesions unaltered. Results of capillaroscopy showed improvement in 5 of 11 cases. Biological values could not be correlated with either the course or the therapeutic efficacy. General tolerance to PE was good but the venous approach must be of good quality. These findings suggest the need for a randomized trial to define the place of PE in the treatment of GS.

Adult↗
PubMed1988

[Pathophysiology of fibroses. Progressive systemic scleroderma as a model disease].

Fibrosis is characterized by excessive deposition of connective tissue in the involved organs. Although the prime event in the pathogenesis is still poorly understood, several mechanisms are discussed, which finally result in the activation of fibroblasts. Recent studies have demonstrated that immunocompetent cells play a crucial role during the initial phases of the development of fibrosis. Therefore, several mediators have been characterized, which are secreted by platelets, lymphocytes and macrophages and which can attract fibroblasts, induce proliferation and collagen synthesis in mesenchymal cells. These include PDGF, EGF, TGF--beta and many others. In addition, gamma-interferon has been shown to inhibit chemotaxis of fibroblasts and to reduce collagen mRNA levels. A controlled interaction of all the different mediators is required to guarantee a normal functioning of connective tissue. Alterations in these regulation steps can result in excessive deposition of connective tissue and the development of fibrotic processes.

Collagen↗