@ERSpublicationsWe should be aware that low DLCO in a patient presenting with PAH may signal another disease and a dismal prognosis http://ow.ly/tvK6wThe increased interest in pulmonary arterial hypertension (PAH) witnessed during the last decades has dramatically changed our comprehension of the disease and its management, resulting in better functional capacity and haemodynamic profiles, and increased survival [1,2]. The discovery of different pathophysiological pathways associated with PAH progression has allowed the development of targeted therapies that were responsible for such improvements [3].Recently, more attention has been paid to the epidemiology and distinct phenotypes of PAH, not least by publication of several registries expanding our knowledge on the clinical and demographic characteristics of PAH patients [4][5][6][7][8][9] as well as on the burden that previously unrecognised forms of PAH might represent [10]. The pivotal study conducted by the US National Institutes of Health in the 1980s [11,12] provided invaluable information about the natural course of PAH. The patients in that cohort were predominantly young females (mean age of 36 years) presenting with what was then called primary pulmonary hypertension, i.e. idiopathic, familial or appetite suppressant-associated PAH. The presence of comorbidities did not represent a significant issue at that time, and patients seemed to present with pure vascular disease and few comorbidities. Recent registries have shown a considerable shift in this scenario [4][5][6][7][8][9]. Particularly in the western world, the majority of PAH patients are now diagnosed at older age (50-60 years or even older) with a significant increase in comorbidities not necessarily, but possibly, associated with the development of pulmonary hypertension. The UK and Ireland registry recently described a subgroup of newly diagnosed PAH patients .50 years of age with increased prevalence of obesity, systemic hypertension, diabetes and ischaemic heart disease, presenting with a worse prognosis, despite a seemingly better haemodynamic profile at baseline [6]. Nonetheless, some of these comorbidities may also cause pulmonary hypertension, hence increasing the complexity in diagnosing PAH [13]. Most of the current classification system for pulmonary hypertension is based on the baseline clinical condition and haemodynamic profile of the patient [14]. In this sense, coexistence of multiple clinical conditions such as mild emphysema or pulmonary fibrosis, or even mild heart failure with preserved ejection fraction, which is commonly seen in the elderly, might complicate the appropriate diagnosis. Thus, we need better diagnostic markers to define and distinguish the various phenotypes of pulmonary vascular disease.Diffusing capacity of the lung for carbon monoxide (DLCO) is one of these potential markers thanks to its characteristic of reflecting alveolar and microvascular integrity [15]. For many years, a low DLCO has been associated with the development of PAH in patients with syste...