How Does Scleroderma Renal Crisis Develop?
The events that lead to this crisis are imperfectly understood, but appear to involve at first constriction of blood vessels within the kidney, followed by actual scarring of these vessels with growth of the lining cells of blood vessels (endothelial cells and smooth muscle cells), and deposition of fibrin, the material that forms blood clots, inside the vessel. The process tends to involve medium-sized blood vessels in the kidney. As a result of impaired blood flow in the kidney, kidney hormones are released that bring on a cascade of blood vessel constriction and further impaired blood flow. There may be injury or actual death of parts of the kidney, both as a result of the initial process and the ensuing severely high blood pressure. The high blood pressure and constricted and occluded (blocked) blood vessels present an impediment to normal heart function, and some component of heart failure is common. Because blood vessels are literally occluded, red blood cells are sheared as they