Τίτλος:
Pulmonary hypertension in patients with interstitial lung disease
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
Interstitial lung diseases (ILDs) comprise a broad and heterogeneous group of more than two hundred diseases with common functional characteristics. Their diagnosis and management require a multidisciplinary approach. This multidisciplinary approach involves the assessment of comorbid conditions including pulmonary hypertension (PH) that exerts a dramatic impact on survival. The current World Health Organization (WHO) classification of PH encompasses many of the interstitial lung diseases into WHO Group 3, while sarcoidosis, Pulmonary Langerhans Cell Histiocytosis and lymphangioleiomyomatosis are placed into WHO Group 5 as diseases with unclear or multifactorial mechanisms. Connective tissue diseases could span any of the 5 WHO groups based on the primary phenotype into which they manifest. Interestingly, several challenging phenotypes present with features that overlap between two or more WHO PH groups. Currently, PH-specific treatment is recommended only for patients classified into WHO Group 1 PH. The lack of specific treatment for other groups, including PH in the setting of ILD, reflects the poor outcomes of these patients. Thus, identification of the optimal strategy for ILD patients with PH remains an amenable need. This review article provides a brief overview of biomarkers indicative of vascular remodeling in interstitial lung disease, summarizes the current state of knowledge regarding patients with PH and ILD and highlights future perspectives that remain to be addressed. © 2018 Elsevier Ltd
Συγγραφείς:
Karampitsakos, T.
Tzouvelekis, A.
Chrysikos, S.
Bouros, D.
Tsangaris, I.
Fares, W.H.
Περιοδικό:
Pulmonary Pharmacology and Therapeutics
Εκδότης:
INSTAP Academic Press
Λέξεις-κλειδιά:
ambrisentan; amino terminal pro brain natriuretic peptide; biological marker; bosentan; chemokine receptor CXCR2; chemokine receptor CXCR2 antagonist; endostatin; gelatinase A; hyaluronic acid; hyaluronic acid binding protein; macitentan; pigment epithelium derived factor; pirfenidone; plasminogen activator inhibitor 1; platelet derived growth factor; prostacyclin; protein tyrosine kinase inhibitor; riociguat; selexipag; sildenafil; tadalafil; tissue plasminogen activator; transforming growth factor beta; treprostinil; vasculotropin; vasculotropin receptor 2, comorbidity; connective tissue disease; disease classification; granulomatous vasculitis; heart right ventricle; human; interstitial lung disease; Langerhans cell histiocytosis; lung vasoconstriction; lymphangioleiomyomatosis; nonhuman; phenotype; priority journal; pulmonary artery; pulmonary hypertension; Review; sarcoidosis; scleroderma; treatment response; vascular remodeling; interstitial lung disease; pathology; pathophysiology; pulmonary hypertension, Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial; Pulmonary Artery
DOI:
10.1016/j.pupt.2018.03.002
