Response to pulmonary vasodilator treatment in a former smoker with combined interstitial lung disease complicated by pulmonary hypertension: Case report and review of the literature

Mercurio, Valentina; Carlomagno, Guido; Fazio, Serafino

doi:10.1016/j.hrtlng.2011.09.005

Cited by 16 publications

(9 citation statements)

References 19 publications

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“…Transthoracic echocardiography is not accurate on the estimation of systolic Ppa especially in patients with lung diseases [76] and right heart catheterization (RHC) should be performed in cases where the accurate measurement of pulmonary haemodynamics is necessary [71] for example in cases where specific treatment is considered. There are no specific recommendations regarding the treatment of PH in patients with CPFE although several case reports indicate that specific therapy for PH may improve haemodynamics, in these patients [73,77,78] but without clear evidence for clinical and survival benefit.…”

Section: Pulmonary Hypertensionmentioning

confidence: 99%

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Combined pulmonary fibrosis and emphysema: The many aspects of a cohabitation contract

Papaioannou

Κostikas

Manali

et al. 2016

Respiratory Medicine

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Combined pulmonary fibrosis and emphysema (CPFE) is a clinical entity characterized by the coexistence of upper lobe emphysema and lower lobe fibrosis. Patients with this condition experience severe dyspnea and impaired gas exchange with preserved lung volumes. The diagnosis of the CPFE syndrome is based on HRCT imaging, showing the coexistence of emphysema and pulmonary fibrosis both in varying extent and locations within the lung parenchyma. Individual genetic background seem to predispose to the development of the disease. The risk of the development of pulmonary hypertension in patients with CPFE is high and related to poor prognosis. CPFE patients also present a high risk of lung cancer. Mortality is significant in patients with CPFE and median survival is reported between 2.1 and 8.5 years. Currently, no specific recommendations are available regarding the management of patients with CPFE. In this review we provide information on the existing knowledge on CPFE regarding the pathophysiology, clinical manifestations, imaging, complications, possible therapeutic interventions and prognosis of the disease.

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Section: Pulmonary Hypertensionmentioning

confidence: 99%

“…In patients with airflow obstruction inhaled bronchodilators might be also prescribed [16]. In the presence of pulmonary hypertension, specific therapy might result to the improvement of haemodynamics [73,77,78] although the use of these therapies in CPFErelated pulmonary hypertension needs to be further evaluated.…”

Section: Therapeutic Interventionsmentioning

confidence: 99%

Combined pulmonary fibrosis and emphysema: The many aspects of a cohabitation contract

Papaioannou

Κostikas

Manali

et al. 2016

Respiratory Medicine

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“…Patients with CPFE are likely to require long-term oxygen therapy. The presented case indicates that therapy specific for pulmonary hypertension may improve haemodynamics, as in other isolated reports [13,16], but the potential clinical and survival benefit is unknown. Results of a recent trial (‘ARTEMIS-IPF‘, NCT00768300) have showed that ambrisentan therapy is not beneficial and may even be deleterious in patients with IPF, regardless of associated PH, and should now be avoided in this condition.…”

Section: Discussionmentioning

confidence: 54%

Clinical case: Combined pulmonary fibrosis and emphysema with pulmonary hypertension – clinical management

Cottin

2013

BMC Res Notes

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BackgroundCombined idiopathic pulmonary fibrosis (IPF) with pulmonary emphysema (CPFE) is a syndrome with a characteristic presentation of upper lobe emphysema and lower lobe fibrosis. While CPFE is a strong determinant of secondary precapillary pulmonary hypertension (PH), there is limited evidence regarding the management of patients with CPFE and PH.Case presentationA 63 year-old male presented in 2006 with dyspnoea on exertion having quit smoking in 2003. Clinical examination, together with high resolution computed tomography, bronchoalveolar lavage, and echocardiographic assessments, suggested a diagnosis of CPFE without PH. In 2007, the patient received intravenous cyclophosphamide, N-acetylcysteine, and short-term anticoagulation treatment. Due to remission of acute exacerbations, the patient received triple combination therapy (prednisone, N-acetylcysteine and azathioprine). Upon progressive clinical worsening, long-term supplemental oxygen therapy was initiated in 2009. Repeated right heart catheterisation in 2011 confirmed PH and worsening pulmonary haemodynamics, and off-label ambrisentan therapy was initiated. Dyspnoea remained at follow-up, although significant haemodynamic improvement was observed.ConclusionCFPE is a distinct but under-recognized and common syndrome with a characteristic presentation. Further studies are needed to ascertain the etiology, morbidity, and mortality of CPEF with or without PH, and to evaluate novel management options.

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“…The importance of identifying this condition lies in the fact that up to 35% of patients with IPF may actually have CPFE and thus may have a different clinical course than patients with IPF alone (2). These patients depend on compensatory hypoxic vasoconstriction to modify their ventilation/ perfusion mismatch and attempt to preserve normoxemia which puts them at risk of having a paradoxical hypoxemic response to commonly utilized pulmonary vasodilator therapies with prostacyclin analogues, endothelin receptor antagonists and phosphodiesterase inhibitors aimed at treating PH in such patients (7). Patients with CPFE are at a high risk of developing lung cancer and require annual surveillance CT scans (8) and also need to be referred for lung transplant earlier than somebody with similar degree of emphysema or fibrosis alone.…”

Section: Discussionmentioning

confidence: 99%

The worst of both worlds-combined pulmonary fibrosis and emphysema syndrome

Aqeel¹,

Biswas²,

Pothapregada³

2016

Annals of Translational Medicine

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Response to pulmonary vasodilator treatment in a former smoker with combined interstitial lung disease complicated by pulmonary hypertension: Case report and review of the literature

Cited by 16 publications

References 19 publications

Combined pulmonary fibrosis and emphysema: The many aspects of a cohabitation contract

Combined pulmonary fibrosis and emphysema: The many aspects of a cohabitation contract

Clinical case: Combined pulmonary fibrosis and emphysema with pulmonary hypertension – clinical management

The worst of both worlds-combined pulmonary fibrosis and emphysema syndrome

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