M. BASHAR KAHALEH, M.D.; IRENE OSBORN, M.S.; E. CARWILE LeROY, M.D.
Von Willebrand factor activity and factor VIII/von Willebrand factor (fVIII/vWf) antigen concentrations were evaluated in 17 patients with scleroderma, nine patients with Raynaud's phenomenon, and eight control volunteers. Higher circulating levels of both activities were seen: von Willebrand factor, 374% ± 40% (percent of control values) in scleroderma patients, 502% ± 104% in patients with Raynaud's phenomenon and 102% ± 6% in control subjects (p < 0. 005, scleroderma versus control); fVIII/vWf antigen, 255% ± 24% in scleroderma patients; 271% ± 46% in patients with Raynaud's phenomenon, and 99% ± 4% in control subjects (p < 0.005, scleroderma versus control). Because endothelial cells synthesize and secrete both substances, the role of endothelial injury in vitro was investigated. Wound injury induced a 344% ± 33% increase in von Willebrand factor and a 115% ± 5% increases in fVIII/vWf antigen; cold injury induced 644% ± 66% and 150% ± 10% increases, and cytotoxic endothelial injury induced 1055% ± 83% and 185% ± 20% increases. In five patients with scleroderma, cold exposure led to a further increase in both activities. The observed increase of both activities in scleroderma and Raynaud's phenomenon may reflect in-vivo endothelial injury and regeneration in these related conditions.
KAHALEH MB, OSBORN I, LeROY EC. Increased Factor VIII/von Willebrand Factor Antigen and von Willebrand Factor Activity in Scleroderma and in Raynaud's Phenomenon. Ann Intern Med. ;94:482–484. doi: 10.7326/0003-4819-94-4-482
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Published: Ann Intern Med. 1981;94(4_Part_1):482-484.
Cardiology, Rheumatology, Scleroderma.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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