Pulmonary hypertension in autoimmune rheumatic diseases

Abstract

Arterial pulmonary hypertension (PH) might be a complication of some autoimmune rheumatic diseases, specially systemic sclerosis. This form of arterial PH is indistinguishable from primary PH, characterised by the presence of plexiform lesions. Although for many years plexiform lesions have been considered end-stage scarring lesions, they are composed by actively proliferating endothelial cells that share many features with cancer cells. Endothelial cells within plexiform lesions in all forms of arterial PH show a decrease in the expression of vasodilator and anti-proliferative factors, and an increase in the expression of vasoconstrictor and angiogenic and mitogenic factors. These cells also show important alterations in growth and apoptosis key regulatory genes. Plexiform lesions are surrounded by inflammatory cell infiltrates, probably providing cytokines that may contribute to the endothelial cell proliferative process. All these data suggest that arterial PH might be seen as a proliferative endothelial cell process, which would open new therapeutic approaches for this devastating disease.