H. Zachariae et al., PLASMA ENDOTHELIN AND THE AMINOTERMINAL PROPEPTIDE OF TYPE-III PROCOLLAGEN (PIIINP) IN SYSTEMIC-SCLEROSIS, Acta dermato-venereologica, 74(5), 1994, pp. 368-370
Forty-four patients with systemic sclerosis and 3 patients with locali
zed scleroderma were investigated for plasma endothelin and aminotermi
nal propeptide of type III procollagen (PIIINP). Although there was an
overlap between plasma levels of endothelin in patients with systemic
sclerosis and healthy controls, the mean value of the patients was si
gnificantly higher than in controls. Plasma endothelin was normal in a
ll 3 patients with localized scleroderma. The highest levels of plasma
endothelin were found in patients with type II and III systemic scler
osis with the largest cutaneous involvement, and in patients with the
scleroderma-specific antibodies Scl-70 and anticentromere antibodies.
Extremely high values were found in a patient who experienced a renal
crisis and in a patient who had her lower leg amputated due to severe
vasculitis. A positive correlation was found between plasma endothelin
and serum PIIINP. This, together with the fact that in systemic scler
osis the vascular lesions are the earliest, would seem to support the
theory that endothelial cell damage could lead to increased secretion
of endothelin and subsequent fibrosis in this disease.