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Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary hypertension is a relevant interceding morbidity in patients with pulmonary fibrosis that has significant impact on exercise tolerance and outcome. The aim of this study was to further characterize the exercise intolerance, dyspnoea and ventilatory inefficiency of patients with pulmonary fibrosis in the presence or absence of pulmonary hypertension via cardiopulmonary exercise testing. Thirty-four patients underwent pulmonary function testing, symptom-limited exercise testing on a bicycle and dyspnoea evaluation according to the BORG scale. Pulmonary hypertension was assessed by echocardiography and in a subset of patient's right heart catheterization. Sixteen of 34 patients with pulmonary fibrosis revealed pulmonary hypertension. While all study patients did not differ in lung functions and demographic characteristics, patients suffering from pulmonary hypertension showed a significantly impaired exercise tolerance and worsened ventilatory inefficiency. The extent of pulmonary artery pressure elevation impacted significantly on ventilatory inefficiency. In addition, the increased ventilatory requirements significantly influenced the extent of dyspnoea in patients with pulmonary hypertension. We conclude that pulmonary hypertension has a significant impact on exercise capacity and dyspnoea in patients with interstitial lung disease (ILD). The further impairment of exercise capacity as well as the extent of dyspnoea in patients with interceding PHT is attributable to a significantly impaired ventilatory inefficiency.
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PMID:Impact of pulmonary hypertension on gas exchange and exercise capacity in patients with pulmonary fibrosis. 1880 60

Pulmonary hypertension (PH) is a common complication of connective tissue disease. While there are no reliable epidemiological data, the prevalence of systemic sclerosis (SSc) has been estimated as being 8-12%. Mixed connective tissue disease (MCTD) and systemic lupus erythematodes (SLE) are thought to have a lower prevalence. PH associated with SSc has a poor prognosis, a two-year survival rate of approximately 50%, if untreated. Systematic literature search for studies of PH and CTD between 02/2007 and 02/2008 found 38 articles, a selection of which is reviewed here. One epidemiological study showed that nowadays PH is together with interstitial pulmonary fibrosis the most common cause of death in patients with SSc. Before the introduction of angiotensin- converting enzyme inhibitors the most frequent cause of death was acute renal crisis. Investigations of the pathogenesis of PH in CTD revealed that in patients with a severe, treatment-resistant course there is frequent histological evidence of pulmonary veno-occlusive disease. A prospective study on diagnosis revealed that transthoracic echocardiography (TTE) is better than magnetic resonance imaging or pulmonary function tests especially in screening for PH in patients with SSc, because of its high specificity and good predictive value at higher pulmonary pressures. But because of the low sensitivity of TTE right heart catheterization is the gold standard for verifying PH also in patients with SSc. A therapeutic uncontrolled trial indicated that treatment with bosentan combined with sildenafil results in clinical stabilization, but patients with idiopathic PHT responded better.
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PMID:[Update in pulmonary hypertension associated with connective tissue diseases - a systematic literature review]. 1881 96