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V Steen

Publications and source records attributed to V Steen.

4 recordsLinked to original sources

Lower extremity amputation in scleroderma.

Scleroderma or Systemic Sclerosis (SSC) is a disorder characterized by fibrosis of the skin and multiple internal organs. The pathological lesion is a triad of small artery intimal proliferation, medial thinning and adventitial scarring. Autoamputation of fingers and toes is often seen, but only a few cases of limb amputation in scleroderma patients have been reported. The Pittsburgh Scleroderma databank includes 1,030 patients with SSC. Among these were seven patients who sustained lower limb amputation. There were four patients with the CREST variant of SSC, two with diffuse scleroderma, and one who had SSC/rheumatoid arthritis/polymyositis overlap who sustained limb amputation. Of the seven, three were male and five had a significant smoking history. Ages ranged from 46 to 71 years. All patients underwent amputation for nonhealing ulcerations. No problems with postoperative wound healing were seen. Pathologic changes typical of SSC in addition to atherosclerotic peripheral vascular disease were described in one case. Three patients were successfully fitted with prostheses and became independent ambulators. Four patients could not be fitted with prostheses. No skin problems were reported related to prosthetic use. Our review demonstrates that SSC patients who undergo amputation can become successful prosthetic users and should be considered for prosthetic prescription.

Aged

Treatment of systemic sclerosis.

The last few years have brought a resurgence in interest in managing systemic sclerosis. In the past year, there have been reports of small series of patients and several well-designed, double-blind, controlled trials. Treatment has been directed at a variety of potential pathogenic mechanisms. Stanazol, iloprost, plasmapheresis, thyroxine, and calcitonin were felt to have positive effects on vascular aspects of disease, ie, Raynaud's phenomenon. Immune-mediated treatment using antithymocyte and antilymphocyte globulin, cyclosporine, and methotrexate were encouraging in a small number of patients, but controlled studies of plasma exchange, extracorporeal phototherapy, and 5-fluorouracil were not very exciting. Changing fibroblast function was used with some success in some patients with D-penicillamine and interferon gamma, but not ketotifen. A very dramatic improvement in the survival of renal crisis occurred with the use of angiotensin-converting enzyme inhibitor. Hopefully, this improvement in survival will also occur in the other visceral abnormalities. All this activity in the therapy of systemic sclerosis will certainly lead to improvement in the overall management of disease.

Calcitonin

Functional and phenotypic analysis of T lymphocytes cloned from the skin of patients with systemic sclerosis.

Activated T lymphocytes often accumulate in the lower dermis of patients with systemic sclerosis (scleroderma) and may play a role in the development of dermal fibrosis. We propagated and cloned these cells directly from skin biopsies in four of eight cases of early, untreated systemic sclerosis with diffuse scleroderma. The cloning frequency estimates were f = 0.20 and f = 0.48 for T cells derived from the skin of two patients versus f = 0.68 and f = 0.96 for autologous blood T lymphocytes. All but one of 24 skin-derived scleroderma clones were CD4+. Clonal analyses performed with CD4+ clones from patients and normal controls showed that all but one skin-derived clones synthesized either interferon-gamma (60%), glycosaminoglycan-stimulatory factor (26%) or both (9%) when induced in vitro by a mitogen, concanavalin A, but not by autologous dermal fibroblasts. In contrast, blood-derived clones had a different functional phenotype. All skin-derived clones produced tumour necrosis factor-alpha. Our results demonstrate that T lymphocytes obtained from the skin of patients with systemic sclerosis synthesized cytokines which could modulate functions of human dermal fibroblasts.

Adult