The Relationship of Hypertension and Renal Failure in Scleroderma (Progressive Systemic Sclerosis) to Structural and Functional Abnormalities of the Renal Cortical Circulation

Abnormalities of Renal Physiology in Systemic Sclerosis

“…Other studies have shown that renal crisis affects 7-18% of SSc patients, largely those with diffuse skin involvement and then, usually within the first 5-6 years of SSc onset (1,3,5,7,25). Renal crisis occurred in only 2 of our patients (4%), both of whom had diffuse SSc: for 6 years in one and for 11 years in the other.…”

“…PAH clearances in normal subjects and xenon-clearances in SSc patients decrease significantly when the test subject's hand or foot is immersed in ice water (1,20,24). In our study, we used an icepack wrapped snugly around the posterior neck and shoulders, a stimulus which Kovalchik found adequate to raise PRA levels in SSc patients (9).…”

Increased serum levels of soluble CD163 in patients with scleroderma

“…Vascular endothelial dysfunction is one of the central events in SSc, and cold-and stress-induced vasospasm (Raynaud's phenomenon) is the most characteristic sign that reflects this dysfunction. Raynaud's phenomenon occurs not only in the fingers and toes but also in the internal organs, such as the kidney [28]. Ischaemia and reperfusion injury following Raynaud's phenomenon can generate reactive oxygen species that may result in vascular endothelial damage [29,30].…”

Scleroderma Renal Crisis

“…If the injury is sufficiently severe, endothelial damage can initiate arteriolar and arterial fibrinoid necrosis and vascular thrombosis through platelet activation and adhesion to the sub-endothelium (Batal, Domsic, Medsger, & Bastacky, 2010;Fisher & Rodnan, 1958;Steen, 2003). With time, these changes can organize leading to fibrointimal thickening and narrowing of the small arteries (Cannon et al, 1974;Fisher & Rodnan, 1958). Intimal arteritis, manifested as lymphocytic and mononuclear cell infiltration, is typically absent in SRC (Steen, 2003).…”