Pulmonary hypertension: advances in pathogenesis and treatment.
Pulmonary hypertension is an orphan disease that until recently has received limited attention within the wider medical community. This has changed distinctly in the last 10 years with the advent of new classes of therapy and a renewed interest in mechanisms of pathogenesis. This review utilized information gathered from recent conferences, and a review of the literature was conducted using MedLine and Pubmed. Accepted mechanisms of pathogenesis and currently available treatments are presented. We will discuss interesting new concepts in pathogenesis, including the importance of genetic forms of the disease and in particular the transforming growth factor receptor superfamily and the evolving evidence of the contribution of dysregulated immunity. Areas of research may yield therapeutic benefits in the not-too-distant future, including anti-proliferative therapies and stem cell therapy.
Toshner
M
M
Department of Medicine, University of Cambridge, Cambridge CB2 2QQ, UK.
Tajsic
T
T
Morrell
N W
NW
D001323:Autoantibodies / Q000378:metabolism; D052006:Bone Morphogenetic Protein Receptors, Type II / Q000235:genetics; D016228:Endothelial Growth Factors / Q000037:antagonists & inhibitors; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000628:therapy*; D007249:Inflammation / Q000276:immunology; D009154:Mutation; D033581:Stem Cell Transplantation; D014665:Vasodilator Agents / Q000627:therapeutic use
D001323:Autoantibodies; D016228:Endothelial Growth Factors; D014665:Vasodilator Agents; D052006:Bone Morphogenetic Protein Receptors, Type II