Thorax 69:107-108 doi:10.1136/thoraxjnl-2013-204964
  • Editorial

Pulmonary hypertension complicating pulmonary fibrosis: bad and ugly, but good to treat?

  1. Steven D Nathan
  1. Correspondence to Dr Steven D Nathan, Advanced Lung Disease and Transplant Program, Department of Medicine, Inova Heart and Vascular Institute, Falls Church, VA 22042, USA; steven.nathan{at}
  • Received 12 December 2013
  • Accepted 13 December 2013

Pulmonary hypertension (PH) often complicates the course of patients with many forms of advanced lung disease, including those with pulmonary fibrosis.1 There is a growing appreciation of the association of PH with greater functional impairment, higher oxygen needs and poorer outcomes.2 What remains unknown is how much of a role PH has in these detrimental outcomes or whether it is an adaptive phenomenon and a surrogate for other untoward consequences of the parenchymal lung disease. Indeed, it is conceivable that, at its outset, PH is adaptive or reactive and in some patients evolves to a maladaptive phenomenon. This two-compartment concept is central to the notion of disproportionate PH, which is often spoken about but remains ill-defined. By implication, PH is ‘disproportionate’ when it is felt unlikely to be explained by the extent of the parenchymal lung disease alone, is functionally limiting, and/or is the primary driver of outcomes. This inevitably leads to the question of whether or not such patients might benefit from pulmonary vasoactive medications.

With the increasing number and availability of approved agents to treat group 1 pulmonary arterial hypertension (PAH), there has been growing interest and curiosity in treating group 3 PH. When weighing the appeal of this strategy, it is important to remember that prior advances in medicine have been punctuated with interventions that made biological and physiological sense, but did not pan out, and in some cases inflicted more harm than good. …