title: Pulmonary hypertension in chronic interstitial lung diseases.
abstract: Pulmonary hypertension (PH) is a common complication of interstitial lung diseases (ILDs), particularly in idiopathic pulmonary fibrosis and ILD associated with connective tissue disease. However, other lung diseases, such as combined pulmonary fibrosis and emphysema syndrome, pulmonary Langerhans cell histiocytosis, and lymphangioleiomyomatosis, may also include PH in their clinical manifestations. In all of these diseases, PH is associated with reduced exercise capacity and poor prognosis. The degree of PH in ILDs is typically mild-to-moderate. However, some of these patients may develop a disproportionate increase in PH that cannot be justified solely by hypoxia and parenchymal injury: this condition has been termed "out-of-proportion" PH. The pathogenesis of PH in these diseases is various, incompletely understood and may be multifactorial. The clinical suspicion (i.e. increased dyspnoea, low diffusion capacity) and echocardiographic assessment are the first steps towards proper diagnosis of PH; however, right heart catheterisation remains the current gold standard for diagnosis of PH. At present, no specific therapies have been approved for the treatment of PH in patients with ILDs.
authors.affiliation: Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe - MultiMedica, Milan, Italy.
authors.forename: Antonella
authors.initials: A
authors.lastname: Caminati
authors.forename: Roberto
authors.initials: R
authors.lastname: Cassandro
authors.forename: Sergio
authors.initials: S
authors.lastname: Harari
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use; D062186:Arterial Pressure / Q000187:drug effects; D006328:Cardiac Catheterization; D002908:Chronic Disease; D004452:Echocardiography; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D008168:Lung / Q000503:physiopathology; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D011237:Predictive Value of Tests; D011651:Pulmonary Artery / Q000187:drug effects / Q000503:physiopathology; D012307:Risk Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents