Figures
- FIGURE 1
Low diffusing capacity of lungs for carbon monoxide (DLCO) in patients with pulmonary vascular disease (PVD) (courtesy of Karen Olssen). IPAH: idiopathic pulmonary arterial hypertension; SSc: systemic sclerosis; PVOD: pulmonary veno-occlusive disease; PCH: pulmonary capillary haemangiomatosis; PAH: pulmonary arterial hypertension.
- FIGURE 2
OPTICS risk score. HDL-C: high-density lipoprotein cholesterol; LDL-C: low-density lipoprotein cholesterol; SV1: s wave in V1 ECG lead; RV6: r wave in V6 ECG lead; PH: pulmonary hypertension.
Tables
- TABLE 1
Overview of trials with targeted pulmonary hypertension (PH) medication in interstitial lung diseases mentioned in this article
Trial Subjects n Study type Drugs Patient population Duration Primary end-point result Other outcomes STEP-IPF (2010) [56] 180 RCT Sildenafil versus placebo 12 weeks No improvement in 6MWT No difference in death or exacerbation
Improvement in QoLDawes et al. (2021) [57] 183 Retrospective PDE-5 versus no treatment Group 3 PH (67% ILD-PH) Median follow-up 1.6 years Transplant-free survival 2.33 versus 1.09 years INSTAGE (2018) [58] 247 RCT Sildefanil+nintedanib versus placebo+nintedanib IPF+PH 24 weeks No improvement in QoL Reduction of death/decline in FVC Behr et al. (2021) [59] 247 RCT Sildenafil+pirfenidone versus placebo+pirfenidone IPF+risk of PH 52 weeks No benefit for disease progression/death Artemis-IPF (2013) [81] 68 RCT Ambrisentan versus placebo IPF Terminated early Lack of efficacy and potential harm (more hospitalisation) RISE-IIP (2019) [61] 147 RCT Riociguat versus placebo IIP+PH Terminated early Increased SAE and death in patients with riociguat INCREASE (2021) [62] 326 RCT Treprostinil inhalative versus placebo ILD-PH 16 weeks Increase in 6MWT Improvement NT-proBNP, time to clinical worsening RCT: randomised controlled trial; 6MWT: 6-min walk test; QoL: quality of life; PDE-5: phosphodiesterase-5; ILD: interstitial lung disease; FVC: forced vital capacity; IPF: idiopathic pulmonary fibrosis; IIP: idiopathic interstitial pneumonia; SAE: serious adverse event; NT-proBNP: N-terminal pro-brain natriuretic peptide.
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- Article
- Abstract
- Abstract
- Pulmonary embolism and related controversies
- Pro–con debate: the current classification of pulmonary hypertension meets clinical practice
- Pulmonary hypertension due to lung diseases and/or hypoxia
- Post-capillary PH
- Portopulmonary hypertension
- Pulmonary hypertension in the COVID-19 pandemic and beyond
- Imaging of pulmonary hypertension in adults: a position paper from the Fleischner Society
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