TY - JOUR T1 - Iron deficiency in pulmonary arterial hypertension: a potential therapeutic target JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/09031936.00037711 SP - erj00377-2011 AU - C.J. Rhodes AU - J. Wharton AU - L. Howard AU - J.S.R. Gibbs AU - A. Vonk-Noordegraaf AU - M.R. Wilkins Y1 - 2011/04/08 UR - http://erj.ersjournals.com/content/early/2011/05/18/09031936.00037711.abstract N2 - Iron deficiency is known to be common and detrimental in chronic left heart failure, where parenteral iron treatment has been shown to improve exercise capacity, NYHA functional class and patient well being. There is now increasing interest in the role of iron in the natural history of pulmonary arterial hypertension (PAH). Iron availability influences the pulmonary vasoconstrictor response to hypoxia and accumulating evidence indicates that iron deficiency is prevalent in idiopathic and heritable forms of PAH, iron status being related to exercise capacity, symptoms and poorer survival in patients with idiopathic PAH. Potential mechanisms behind iron deficiency in idiopathic PAH including inhibition of dietary iron uptake by the master iron regulator hepcidin, are discussed. High hepcidin levels underlie the anaemia of chronic disease. Possible stimuli of the observed high levels of hepcidin in IPAH include dysfunctional bone morphogenetic protein receptor type II (BMPR2) signalling and inflammation. Iron status may influence outcomes through modulation of the pulmonary circulation as well as myocardial and skeletal muscle function. Two parallel studies, from our centre and others in the UK and Amsterdam, investigating the safety and potential benefit of iron supplementation in patients with PAH, are currently underway. ER -