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Background: Animal studies and data from a single-center study suggest that tobacco smoke exposure may be a risk factor for precapillary pulmonary hypertension (PH). Objective: We aimed to survey tobacco smoke exposure in a large PH collective and to compare it with epidemiological data from healthy subjects. Methods: This is an international, multicenter, case-control study including patients with pulmonary arterial and chronic thromboembolic PH. All patients were asked specific questions about tobacco smoke exposure. Healthy controls were retrieved from the Swiss Health Survey (n = 18,747). Results: Overall (n = 472), 49% of PH patients were smokers and there was a clear sex difference (women 37%, men 71%). Significantly more PH men were smokers compared with healthy controls, whereas less PH women were ever active smokers. However, 50% of the non-smoking PH women were exposed to secondhand smoke, leading to a significantly higher number of tobacco smoke-exposed individuals compared to healthy controls. PH smokers were significantly younger compared to those not exposed. Conclusion: Active and environmental tobacco smoke exposure is common in PH. The higher prevalence of male PH smokers, the higher exposure to environmental tobacco smoke in PH women compared to healthy controls and the lower age at PH diagnosis in smokers may indicate a pathogenic role of tobacco smoke exposure in PH.
Riociguat is one of several approved therapies available for patients with pulmonary arterial hypertension (PAH). Treatment should be initiated and monitored at an expert center by a physician experienced in treating PAH, and the dose adjusted in the absence of signs and symptoms of hypotension. In certain populations, including patients with hepatic or renal impairment, the elderly, and smokers, riociguat exposure may differ, and dose adjustments should therefore be made with caution according to the established scheme. Common adverse events are often easily managed, particularly if they are discussed before starting therapy. Combination therapy with riociguat and other PAH-targeted agents is feasible and generally well tolerated, although the coadministration of phosphodiesterase type 5 inhibitors (PDE5i) and riociguat is contraindicated. An open-label, randomized study is currently ongoing to assess whether patients who do not achieve treatment goals while receiving PDE5i may benefit from switching to riociguat. In this review, we provide a clinical view on the practical management of patients with PAH receiving riociguat, with a focus on the opinions and personal experience of the authors.
The reviews of this paper are available via the supplemental material section.
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease which is often
caused by recurrent emboli. These are also frequently found in patients with myeloproliferative
diseases. While myeloproliferative diseases can be caused by gene defects, the genetic predisposition
to CTEPH is largely unexplored. Therefore, the objective of this study was to analyse these genes
and further genes involved in pulmonary hypertension in CTEPH patients. A systematic screening
was conducted for pathogenic variants using a gene panel based on next generation sequencing.
CTEPH was diagnosed according to current guidelines. In this study, out of 40 CTEPH patients
4 (10%) carried pathogenic variants. One patient had a nonsense variant (c.2071A>T p.Lys691*)
in the BMPR2 gene and three further patients carried the same pathogenic variant (missense variant,
c.1849G>T p.Val617Phe) in the Janus kinase 2 (JAK2) gene. The latter led to a myeloproliferative
disease in each patient. The prevalence of this JAK2 variant was significantly higher than expected
(p < 0.0001). CTEPH patients may have a genetic predisposition more often than previously thought.
The predisposition for myeloproliferative diseases could be an additional risk factor for CTEPH
development. Thus, clinical screening for myeloproliferative diseases and genetic testing may be
considered also for CTEPH patients.