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J Exp Med. 1979 Jun 1; 149(6): 1326–1335.
PMCID: PMC2184886

Endothelial injury in scleroderma


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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Selected References

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  • LeRoy EC. Increased collagen synthesis by scleroderma skin fibroblasts in vitro: a possible defect in the regulation or activation of the scleroderma fibroblast. J Clin Invest. 1974 Oct;54(4):880–889. [PMC free article] [PubMed]
  • Campbell PM, LeRoy EC. Pathogenesis of systemic sclerosis: a vascular hypothesis. Semin Arthritis Rheum. 1975 May;4(4):351–368. [PubMed]
  • Norton WL, Nardo JM. Vascular disease in progressive systemic sclerosis (scleroderma). Ann Intern Med. 1970 Aug;73(2):317–324. [PubMed]
  • Sinclair RA, Antonovych TT, Mostofi FK. Renal proliferative arteriopathies and associated glomerular changes: a light and electron microscopic study. Hum Pathol. 1976 Sep;7(5):565–588. [PubMed]
  • Jaffe EA, Nachman RL, Becker CG, Minick CR. Culture of human endothelial cells derived from umbilical veins. Identification by morphologic and immunologic criteria. J Clin Invest. 1973 Nov;52(11):2745–2756. [PMC free article] [PubMed]
  • Böyum A. Isolation of mononuclear cells and granulocytes from human blood. Isolation of monuclear cells by one centrifugation, and of granulocytes by combining centrifugation and sedimentation at 1 g. Scand J Clin Lab Invest Suppl. 1968;97:77–89. [PubMed]
  • Haudenschild CC, Zahniser D, Folkman J, Klagsbrun M. Human vascular endothelial cells in culture. Lack of response to serum growth factors. Exp Cell Res. 1976 Mar 1;98(1):175–183. [PubMed]
  • Gimbrone MA, Jr, Cotran RS. Human vascular smooth muscle in culture. Growth and ultrastructure. Lab Invest. 1975 Jul;33(1):16–27. [PubMed]
  • Jaffe EA. Endothelial cells and the biology of factor VIII. N Engl J Med. 1977 Feb 17;296(7):377–383. [PubMed]
  • Fleischmajer R, Perlish JS, Reeves JR. Cellular infiltrates in scleroderma skin. Arthritis Rheum. 1977 May;20(4):975–984. [PubMed]
  • Hüttner I, Boutet M, More RH. Studies on protein passage through arterial endothelium. I. Structural correlates of permeability in rat arterial endothelium. Lab Invest. 1973 Jun;28(6):672–677. [PubMed]
  • Giese J. Renin, angiotensin and hypertensive vascular damage: a review. Am J Med. 1973 Sep;55(3):315–332. [PubMed]
  • Harker LA, Ross R, Glomset JA. The role of endothelial cell injury and platelet response in atherogenesis. Thromb Haemost. 1978 Apr 30;39(2):312–321. [PubMed]
  • Moraes JR, Stastny P. A new antigen system expressed in human endothelial cells. J Clin Invest. 1977 Aug;60(2):449–454. [PMC free article] [PubMed]
  • FISHER ER, RODNAN GP. Pathologic observations concerning the kidney in progressive systemic sclerosis. AMA Arch Pathol. 1958 Jan;65(1):29–39. [PubMed]
  • Stemerman MB, Ross R. Experimental arteriosclerosis. I. Fibrous plaque formation in primates, an electron microscope study. J Exp Med. 1972 Oct 1;136(4):769–789. [PMC free article] [PubMed]
  • Friedman RJ, Moore S, Singal DP. Repeated endothelial injury and induction of atherosclerosis in normolipemic rabbits by human serum. Lab Invest. 1975 Mar;32(3):404–415. [PubMed]
  • Harker LA, Slichter SJ, Scott CR, Ross R. Homocystinemia. Vascular injury and arterial thrombosis. N Engl J Med. 1974 Sep 12;291(11):537–543. [PubMed]
  • Cochrane CG, Koffler D. Immune complex disease in experimental animals and man. Adv Immunol. 1973;16(0):185–264. [PubMed]
  • Caplan BA, Schwartz CJ. Increased endothelial cell turnover in areas of in vivo Evans Blue uptake in the pig aorta. Atherosclerosis. 1973 May-Jun;17(3):401–417. [PubMed]
  • Ross R, Glomset J, Kariya B, Harker L. A platelet-dependent serum factor that stimulates the proliferation of arterial smooth muscle cells in vitro. Proc Natl Acad Sci U S A. 1974 Apr;71(4):1207–1210. [PMC free article] [PubMed]
  • Spagnoli LG, Nicosia R, Villaschi S, Cavallero C. Smooth muscle cell response to endothelial injury. Prog Biochem Pharmacol. 1977;13:94–98. [PubMed]
  • Gutstein WH, Parl F. Ultrastructural changes of coronary artery endothelium associated with biliary obstruction in the rat. Am J Pathol. 1973 Apr;71(1):49–60. [PMC free article] [PubMed]
  • Constantinides P, Robinson M. Ultrastructural injury of arterial endothelium. II. Effects of vasoactive amines. Arch Pathol. 1969 Aug;88(2):106–112. [PubMed]
  • Evensen SA, Pickering RJ, Batbouta J, Shepro D. Endothelial injury induced by bacterial endotoxin: effect of complement depletion. Eur J Clin Invest. 1975 Nov 21;5(6):463–469. [PubMed]
  • Minick CR, Alonso DR, Rankin L. Immunologic arterial injury in atherogenesis. Prog Biochem Pharmacol. 1977;13:225–233. [PubMed]

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