Sputum endothelin-1 is increased in cystic fibrosis and chronic obstructive pulmonary disease

Abstract

This study was designed to identify a core set of items that may characterize impaired health in chronic respiratory failure (CRF) since none of the questionnaires commonly used to measure impaired health in patients with CRF were developed for use in such patients.Questionnaire items, identified from several sources, were administered to 92 patients with stable severe chronic hypoxia or hypercapnia, together with physiological measures of disease severity, the Sickness Impact Profile (SIP) and the St George's Respiratory Questionnaire (SGRQ).Of the 152 items identified, only 28 correlated with patients' perceptions of overall health and were also independent of age, sex, disease or type of treatment and did not show floor or ceiling effects. Principal components analysis identified three specific factors: "daily activities", "cognitive function", and "invalidity". The total score for the whole 28-item set correlated with other measures of disease activity, including SGRQ (r=0.86) and SIP (r=0.70), but not spirometry. The interquartile range of the 28-item set was wider than that of the SGRQ or the SIP.This study characterized two areas of health impairment in chronic respiratory failure that had not previously been identified: effects of impaired cognitive function on daily life, and a sense of invalidity. The measurements properties of this 28-item set suggest that it may be a more discriminative instrument for patients with chronic respiratory failure than existing questionnaires.

Pulmonary vascular dysfunction in end-stage cystic fibrosis: role of NF- B and endothelin-1

“…The critical role of ET-1 in PH has been largely demonstrated [20][21][22], underlying the now widely used therapeutic strategies in idiopathic PH based on ET-1 receptor blocking either dually [33,34] or specifically directed to ETAR [35]. Very few data other than increased ET-1 levels in serum [18] and in sputum [19] of CF patients exist as to a potentially pathogenic role of ET-1 in CF. To the best of our knowledge this is the first report on the involvement of ET-1 in CF-associated ED.…”

“…Among these, endothelin (ET)-1 appears relevant in the context of CF. Increased serum levels of ET-1 have been reported in CF patients [18] as well as its airway overproduction [19]. Produced by endothelial cells, this 21-amino acid peptide has very potent vasoconstrictive and mitogenic capacities via signalling through its receptors, ETAR and ETAR.…”

Mediators of Chronic Obstructive Pulmonary Disease

“…There is an increased concentration of endothelin-1 (ET-1) in induced sputum and bronchoalveolar lavage fluid of patients with COPD (Chalmers et al, 1999;Bacakoglu et al, 2003), particularly during exacerbations (Roland et al, 2001). Plasma ET-1 concentrations are also elevated in COPD patients, particularly in patients who develop nocturnal hypoxemia during the night (Trakada et al, 2001;Spiropoulos et al, 2003) and in patients with hypoxemia (Faller et al, 1998), but it is not correlated with secondary pulmonary hypertension (Bacakoglu et al, 2003).…”

Endothelin-1 in stable bronchiectasis

“…mL -1 ), but higher than in those with chronic obstructive pulmonary disease (16.4, 6.8± 38.2 pg . mL -1 ) [15].…”