Vascular Disease in Progressive Systemic Sclerosis (Scleroderma)

Norton, Walter L.

doi:10.7326/0003-4819-73-2-317

Cited by 289 publications

(66 citation statements)

References 51 publications

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“…Connective tissue abnormalities have been explored extensively (1); recently, vascular involvement has been emphasized as a unifying pathogenetic concept (2,3). The vascular features in scleroderma include Raynaud's phenomenon; an early, edematous phase of the disorder; telangiectasia; capillary abnormalities as seen by nailfold and uhrastructural microscopy; and widespread vascular pathology noted in all involved organs.…”

mentioning

confidence: 99%

Endothelial injury in scleroderma.

Kahaleh¹,

Sherer²,

LeRoy³

1979

The Journal of Experimental Medicine

268

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Functional and structural vascular lesions have been observed in the organs involved in scleroderma. The etiology of these vascular changes is poorly understood. The ability to isolate, characterize, and maintain endothelial cells in vitro provides a target cell population to study endothelial damage in scleroderma. The present report describes the effect of scleroderma serum on endothelial, smooth muscle, and fibroblast cell types. Sera from patients with scleroderma (31/52) and Raynaud's syndrome (11/19) contain cytotoxic activity, specific for endothelial cells, which is nondialyzable, heat-stable, and elutes with albumin on gel-filtration chromatography.

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mentioning

confidence: 99%

Endothelial injury in scleroderma.

Kahaleh¹,

Sherer²,

LeRoy³

1979

The Journal of Experimental Medicine

268

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“…Emphasis on the pathogenesis of systemic sclerosis has changed from that of an initial fibrotic process to primary small vessel disease (1,6). Pulmonary hypertension is a common finding in systemic sclerosis (7).…”

Section: Discussionmentioning

confidence: 99%

Vascular reactivity and pulmonary hypertension in systemic sclerosis

Rozkovec

Bernstein

Asherson

et al. 1983

Arthritis & Rheumatism

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“…Scleroderma, whose pathogenesis is likely to involve multiple autoimmune mechanisms, is characterized by a distinctive vascular pathology which is associated with increased collagen formation by fibroblasts, often in a perivascular distribution in the early stages of the disorder [11]. The vascular lesions, which consist of both endothelial cell damage and proliferation of myointimal cells, result in elevated levels of von Willebrand factor antigen [31] and endothelin [32], and are associated with raised levels of -thromboglobulin as an indicator of platelet activation [33].…”

Section: Discussionmentioning

confidence: 99%

“…The development of scleroderma-like lesions as a feature of chronic graft-versus-host disease, both experimentally [9] and in man [10], further supports this concept. A distinctive vascular pathology [11], as well as increased collagen formation by fibroblasts [12], is a characteristic feature of the disease and has led to the recognition by ELISA of anti-endothelial cell antibodies [13], some of which are capable of causing antibody-dependent cellular cytotoxicity (ADCC) [14,15], in the disorder. This study therefore has further characterized antibodies reacting with endothelial and other cell membranes in the disease by immunoblotting sera with membrane and cytosol preparations of human umbilical vein endothelial cells (HUVEC), human dermal fibroblasts and a T cell lymphoma line HUT78.…”

Section: Introductionmentioning

confidence: 99%

Antibodies to membranes of endothelial cells and fibroblasts in scleroderma

Hill¹,

Phipps²,

Cartwright

et al. 1996

Clinical and Experimental Immunology

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SUMMARYAnti-endothelial and other cell membrane-reactive antibodies in scleroderma were characterized by immunoblotting sera with membrane and cytosol preparations of human umbilical vein endothelial cells (HUVEC), dermal fibroblasts and a T cell lymphoma HUT78. Antibodies reactive with HUVEC membranes were found in 17 of 20 patients with scleroderma (33 bands) in contrast to only two of 20 controls (two bands; P<0 . 01) and three of 11 patients with myocardial infarction (four bands). Eleven of the 20 patients possessed antibodies that were specific for HUVEC membrane and did not cross-react with other cell lines. Analysis of patient subgroups showed that HUVEC membrane antibodies were present in nine of 11 patients with systemic sclerosis and in all nine with the CREST syndrome, and were HUVEC-specific in five and six of these cases, respectively. Although considerable heterogeneity was seen, antibodies to an 18-19-kD membrane epitope were found in 11 of the 20 patients but in none of the controls (P<0 . 01). This antibody which reacted particularly with HUVEC (n 9) and HUT78 membranes (n 9) was associated with CREST syndrome rather than systemic sclerosis (9/9 versus 1/ 11; P<0 . 01), and after elution was shown to possess anticentromere activity. In addition, antibodies reactive with both fibroblast (n 11; 18 bands) and HUT78 membranes (n 18; 42 bands) were detected and were specific for either fibroblast or HUT78 membranes in nine and 14 patients, respectively. There was no significant difference in the incidence of these fibroblasts and HUT78 membrane antibodies in the two patient subgroups. These findings support the concept that membranereactive antibodies, including anticentromeric antibodies, may play a central role in the pathogenesis of scleroderma, through their ability to react with endothelial cells.

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Vascular Disease in Progressive Systemic Sclerosis (Scleroderma)

Cited by 289 publications

References 51 publications

Endothelial injury in scleroderma.

Endothelial injury in scleroderma.

Vascular reactivity and pulmonary hypertension in systemic sclerosis

Antibodies to membranes of endothelial cells and fibroblasts in scleroderma

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