Histological analysis of vasculopathy associated with pulmonary hypertension in combined pulmonary fibrosis and emphysema: comparison with idiopathic pulmonary fibrosis or emphysema alone

Awano, Nobuyasu; Inomata, Minoru; Ikushima, Soichiro; Yamada, Daisuke; Hotta, Masatoshi; Tsukuda, Shunji; Kumasaka, Takashi; Takemura, Tamiko; Eishi, Yoshinobu

doi:10.1111/his.13153

Cited by 23 publications

(32 citation statements)

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“…Functionally, abnormal elevations in the ΔTPRex with exercise may reflect an impaired relationship between mPAP and CO due to a limited pulmonary vascular reserve, recruitment and compliance. Independent of the pulmonary vasculature, these abnormalities may also be a consequence of hypoxic pulmonary vasoconstriction during exercise and fibrotic destruction of the pulmonary capillary bed [5,6,[10][11][12][13]. Interestingly, our results suggest that the presence of diabetes mellitus might also be associated with worse outcomes.…”

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confidence: 68%

“…Our study demonstrates that abnormal pulmonary exercise parameters, specifically an abnormal precapillary ΔTPRex, may predict clinical outcomes in patients with fibrotic lung disease. This variable may reflect the underlying pulmonary vascular abnormalities present in some patients with fibrotic lung disease [10][11][12]. Functionally, abnormal elevations in the ΔTPRex with exercise may reflect an impaired relationship between mPAP and CO due to a limited pulmonary vascular reserve, recruitment and compliance.…”

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confidence: 99%

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Exercise pulmonary haemodynamic response predicts outcomes in fibrotic lung disease

et al. 2018

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Pulmonary hypertension is diagnosed by an elevated mean pulmonary arterial pressure (mPAP) during resting right heart catheterisation (RHC) [1]. Although "exercise-induced pulmonary hypertension" is no longer a distinct clinical entity, recently, there has been renewed interest in the prognostic value of pulmonary haemodynamic responses to exercise, particularly in patients with fibrotic lung disease [1-4]. As the optimal use of exercise RHC in this cohort remains unknown, our main objective in this study was to study the relationship between exercise haemodynamics and outcomes in fibrotic lung disease patients [3-7]. All consecutive fibrotic lung disease patients undergoing exercise RHC testing at our centre over a 7-year period, regardless of the mPAP at rest, were included in the study. Fibrotic lung disease, scored by one observer (A. Jose) using the method of GOH et al. [8], was defined as >10% fibrosis on computed tomographic imaging at the time of exercise RHC. Subjects without fibrotic lung disease, those with missing data and those with an elevated pulmonary artery wedge pressure (PAWP) at rest (>15 mmHg) were excluded. Measurements at rest included heart rate, right atrial pressure (RAP), mPAP, digital mean PAWP, thermodilution cardiac output (CO) and pulmonary artery oxygen saturation. Pressures were measured as the digital mean across several respiratory cycles. Pulmonary vascular resistance (PVR) was calculated in the standard fashion. After baseline haemodynamic measurements, the patients underwent supine bicycle ergometer exercise to (subjective) maximal exertion and haemodynamic measurements were repeated. The total pulmonary resistance was calculated both at rest (TPR=mPAP/CO) and at peak exercise (TPRex). The exercise-induced TPR (ΔTPRex=ΔmPAP/ΔCO) was calculated from the difference between rest and peak exercise haemodynamics [1, 3, 5-7]. An abnormal precapillary ΔTPRex, reflecting pulmonary arterial response to exercise, was defined as ΔTPRex ⩾3 mmHg•L −1 •min −1 with concomitant PAWP <20 mmHg at peak exercise. This cutoff was used to definitively exclude patients with occult left heart disease [1, 7]. Demographic, laboratory, functional testing (including 6-min walk test (6MWT)) and pulmonary function testing (PFT) were collected within 3 months of the index exercise RHC. The primary outcome was defined as the first of three clinical worsening events: hospitalisation for cardiopulmonary decompensation, lung transplantation or death. Information on the primary outcome was obtained from the medical record and review of patient's charts. Time zero was the date of the index RHC, and patients were followed until either an event occurred or the study period ended. Patients lost to follow-up were censored at the time of their last clinical encounter. This study was reviewed and approved by the Inova Institutional Review Board (IRB 15-2025). Differences in clinical and haemodynamic characteristics between groups were compared using the Wilcoxon rank-sum test for continuous variables and the Chi-squar...

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Exercise pulmonary haemodynamic response predicts outcomes in fibrotic lung disease

et al. 2018

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“…The most extensive vascular changes were described in the fibrotic areas, less extensive in the emphysematous parts of the lung. Interestingly, some arterial pathology was also found in the areas of preserved lung parenchyma, especially in the patients with CPFE [14].…”

Section: Pathogenesis Of Ph-dpld and Differential Diagnosis Of Ph Caumentioning

confidence: 93%

“…Awano et al [14] have recently reported on the morphology of PA remodelling in the autopsy specimens obtained from the patients with CPFE, IPF and emphysema alone. The most extensive vascular changes were described in the fibrotic areas, less extensive in the emphysematous parts of the lung.…”

Section: Pathogenesis Of Ph-dpld and Differential Diagnosis Of Ph Caumentioning

confidence: 99%

Pulmonary Hypertension in Diffuse Parenchymal Lung Diseases—Is There Any Benefit of PAH-Specific Therapy?

Szturmowicz¹,

Kacprzak²,

Kuś³

2017

Advances in Respiratory Medicine

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Pulmonary hypertension (PH) is diagnosed in 40-50% of the patients with end-stage diffuse parenchymal lung diseases (DPLD), and it is associated with significant worsening of life expectancy. Latest ERS/ESC guidelines recommend best available treatment of DPLD, and long-term oxygen therapy in the patients with PaO 2 less than 60 mm Hg. Pulmonary arterial hypertension (PAH)-targeted drugs are not recommended in PH-DPLD patients, due to the risk of increasing the ventilation-perfusion mismatch, and consequently worsening of hypoxaemia. Nevertheless, PAH-oriented treatment may be beneficial to selected groups of patients. The authors try to find the answer to several important questions: is there any benefit of PAH-specific therapy in PH-DPLD, who should be the candidate for PAH-specific therapy, what class of drugs is most promising, and what outcome measures should be employed?

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“…Whilst it is plausible that PHT in CPFE is not associated with a distinct microvascular phenotype, there might be more to this tale. In a histological analysis of vasculopathy associated with PHT, Awano et al 8 evaluated pulmonary vasculopathy in an autopsy series of patients with CPFE, and compared these findings with those of patients with IPF alone and emphysema alone. Vascular changes were evaluated in the fibrotic areas, emphysematous areas and the relatively unaffected/preserved areas.…”

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Pulmonary hypertension in combined pulmonary fibrosis and emphysema: A tale of two cities

Goh

2018

Respirology

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Histological analysis of vasculopathy associated with pulmonary hypertension in combined pulmonary fibrosis and emphysema: comparison with idiopathic pulmonary fibrosis or emphysema alone

Abstract: These results imply that pulmonary vasculopathy in patients with CPFE could occur in the whole lung tissue. This may explain the tendency for it to lead to the development of pulmonary hypertension in CPFE cases.

Cited by 23 publications

References 32 publications

Exercise pulmonary haemodynamic response predicts outcomes in fibrotic lung disease

Exercise pulmonary haemodynamic response predicts outcomes in fibrotic lung disease

Pulmonary Hypertension in Diffuse Parenchymal Lung Diseases—Is There Any Benefit of PAH-Specific Therapy?

Pulmonary hypertension in combined pulmonary fibrosis and emphysema: A tale of two cities

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