Risk Stratification in Pulmonary Arterial Hypertension, Update and Perspectives.

Risk stratification in pulmonary arterial hypertension (PAH) is crucial in assessing patient prognosis. It serves a prominent role in everyday patient care and can be determined using several validated risk assessment scores worldwide. The recently published 2022 European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines underline the importance of risk stratification not only at baseline but also during follow-up. Achieving a low-risk status has now become the therapeutic goal, emphasising the importance of personalised therapy. The application of these guidelines is also important in determining the timing for lung transplantation referral. In this review, we summarise the most relevant prognostic factors of PAH as well as the parameters used in PAH risk scores and their evolution in the guidelines over the last decade. Finally, we describe the central role that risk stratification plays in the current guidelines not only in European countries but also in Asian countries.

[Hypoxemia: from pathophysiology to diagnosis]. / Hypoxémie : de la physiopathologie au diagnostic.

Hypoxemia is defined as a decreased oxygen partial pressure in arterial blood. This frequent clinical phenomenon can lead to tissue hypoxia and requires a prompt diagnostic approach to guide its management. Five pathophysiological mechanisms should be assessed in the presence of hypoxemia: alveolar hypoventilation, ventilation/perfusion mismatches, diffusion disorders, true shunts and a decrease in the partial pressure of inspired oxygen. In this article, we synthesize the main etiologies of hypoxemia based on respiratory pathophysiology and suggest a diagnostic approach for its evaluation.

L'hypoxémie est définie comme la diminution de la pression partielle en oxygène dans le sang artériel. Ce phénomène fréquent en clinique peut amener à une hypoxie tissulaire et nécessite une approche diagnostique rapide afin d'orienter sa prise en charge. Cinq mécanismes physiopathologiques doivent être évoqués devant une hypoxémie : l'hypoventilation alvéolaire, les inégalités de ventilation/perfusion, les troubles de la diffusion, les shunts vrais et la diminution de la pression partielle d'oxygène inspiré. Dans cet article, nous résumons les étiologies principales d'hypoxémie en se basant sur la physiopathologie respiratoire et proposons une démarche diagnostique pour son évaluation.

[Pulmonary fibrosis and lung cancer: between complementarities and specificities]. / Fibrose pulmonaire et cancer pulmonaire : entre complémentarités et spécificités.

Lung cancer is the leading cause of cancer mortality in the developed world. Diffuse fibrosing interstitial lung disease (ILD) consist of a heterogeneous group that includes idiopathic pulmonary fibrosis (IPF). Diffuse ILD is a risk factor for the development of lung cancer which on its own is associated with an increased risk of morbidity and mortality. Despite common mechanisms between fibrogenesis and carcinogenesis, the underlying pathogenesis of lung cancer and fibrosis overlap is poorly understood. The clinical management of these patients remains a medical challenge requiring a multidisciplinary approach, particularly in view of the risk of acute exacerbation of fibrosing ILD following most lung cancer treatments, leading to a considerable negative outcome on overall prognosis.

Le carcinome bronchopulmonaire (CBP) est la première cause de mortalité par cancer dans les pays développés. Les pneumopathies interstitielles diffuses fibrosantes (PIDf) sont un groupe hétérogène comprenant, entre autres, la fibrose pulmonaire idiopathique. Les PIDf sont un facteur de risque de développement du CBP et sont associées à un risque accru de morbi-mortalité. Malgré des mécanismes communs entre la fibrogenèse et la carcinogenèse, la pathogenèse sous-jacente de l'association CBP et PIDf est mal comprise. La gestion clinique de ces patients est un défi médical nécessitant une prise en charge multidisciplinaire, en particulier devant les risques d'exacerbation aiguë de la PIDf grevant le pronostic.

Comparison of Risk Stratification Scores in Pulmonary Arterial Hypertension: A Monocentric Retrospective Study at Lausanne University Hospital.

BACKGROUND: Risk assessment is the cornerstone of pulmonary arterial hypertension (PAH) management. Risk stratification scores predict prognosis and help individualize treatment. OBJECTIVES: The aims of the study include the following: (1) to compare the prediction for transplant-free survival (TFs) of 3 risk assessment tools at 3 and 5 years after diagnosis and (2) to analyze whether the initial risk stratification was altered after 1 year of treatment. METHOD: We collected retrospectively data of 50 patients diagnosed with PAH Group 1. We categorized them as low, intermediate, and high mortality risk at baseline and at 1 year with the (1) Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) risk score version 2.0, (2) Swedish/Comparative Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (PH) (COMPERA) score, and (3) French PH Network Registry (FPHR) score. RESULTS: TFs at 3 years is predicted by the 3 scores computed at baseline with an area under the curve (AUC) of 0.73, 0.73, and 0.77, respectively. The predictive value increased when the scores were calculated after 1 year of treatment (AUC = 0.91, 0.89, and 0.78). The prediction of TFs at 5 years was better evaluated by the COMPERA and FPHR (AUC = 0.85) than by REVEAL 2.0 (AUC = 0.69) computed at baseline. A low risk status was associated with excellent TFs whatever the scoring used. CONCLUSION: In accordance with the original publications, the 3 scores are able to predict survival up to 5 years after diagnosis. The better performance of the scores after 1 year is a further evidence for their clinical use and an indirect proof for treatment efficacy.

Biologic Treatments in Interstitial Lung Diseases.

Interstitial lung diseases (ILD) represent a group of heterogeneous parenchymal lung disorders with complex pathophysiology, characterized by different clinical and radiological patterns, ultimately leading to pulmonary fibrosis. A considerable proportion of these disease entities present with no effective treatment, as current therapeutic regimens only slow down disease progression, thus leaving patients, at best case, with considerable functional disability. Biologic therapies have emerged and are being investigated in patients with different forms of ILD. Unfortunately, their safety profile has raised many concerns, as evidence shows that they might cause or exacerbate ILD status in a subgroup of patients. This review article aims to summarize the current state of knowledge on their role in patients with ILD and highlight future perspectives.