PAH is a late complication of scleroderma (SSc) in 8 to 10 percent of cases. People with cutaneous scleroderma are at a greater risk of developing PAH than those with diffused cutaneous scleroderma. It so happens that the endothelial cells in the inner lining of the blood vessels are injured and the connective tissue is laid down inside the walls of these blood vessels. The time taken for the development of PAH in SSc patients varies between five to 10 years after the first onset of SSc symptoms.
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