Recent advances in the pharmacotherapy of pulmonary hypertension: practical considerations

Lareo, Ana; Nuche, Jorge; Ropero, María José Cristo; Arribas, Fernando; Oliver, Eduardo; Escribano, Pilar

doi:10.33963/kp.15928

Cited by 7 publications

(3 citation statements)

References 49 publications

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“…PDE5 inhibitors such as sildenafil and tadalafil increase the intracellular cyclic guanosine monophosphate (c-GMP) level by inhibiting PDEs. C-GMP leads to vasodilation through vascular smooth muscle relaxation, increases apoptosis, and decreases proliferation of pulmonary artery smooth muscle cells [ 122 ]. PDE-5 inhibitors have been found to be beneficial in treatment of Raynaud phenomenon, digital ulcers, and pulmonary arterial hypertension [ 123 ].…”

Section: Treatment Strategies For Endothelial Dysfunctionmentioning

confidence: 99%

Endothelial Dysfunction in Systemic Sclerosis

Patnaik,

Lyons,

Tran

et al. 2023

IJMS

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Systemic sclerosis, commonly known as scleroderma, is an autoimmune disorder characterized by vascular abnormalities, autoimmunity, and multiorgan fibrosis. The exact etiology is not known but believed to be triggered by environmental agents in a genetically susceptible host. Vascular symptoms such as the Raynaud phenomenon often precede other fibrotic manifestations such as skin thickening indicating that vascular dysfunction is the primary event. Endothelial damage and activation occur early, possibly triggered by various infectious agents and autoantibodies. Endothelial dysfunction, along with defects in endothelial progenitor cells, leads to defective angiogenesis and vasculogenesis. Endothelial to mesenchymal cell transformation is another seminal event during pathogenesis that progresses to tissue fibrosis. The goal of the review is to discuss the molecular aspect of the endothelial dysfunction that leads to the development of systemic sclerosis.

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Section: Treatment Strategies For Endothelial Dysfunctionmentioning

confidence: 99%

Endothelial Dysfunction in Systemic Sclerosis

Patnaik,

Lyons,

Tran

et al. 2023

IJMS

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“…Current and upcoming preclinical and clinical research is focused on the development of compounds that directly act in the different molecular pathways associated with the development of PAH to counteract the obstructive vascular remodeling and small vessel loss [ 56 , 88 , 89 ]. Specifically, drugs targeting various pathogenic mechanisms are being developed: bone morphogenetic protein signaling, tyrosine kinase receptors, serotonin metabolism, angiogenesis, extracellular matrix, estrogens or epigenetics [ 51 , 90 , 91 ]. Significant scientific advances in preclinical research including new molecular tools, omics technologies, human tissue biobanks and in vivo models, have served to better understand the pathobiology and molecular mechanisms involved in PAH, to develop and test new molecular compounds as adjunctive treatment of PAH that can be subsequently validated in clinical trials (translational research).…”

Section: New Molecular Compounds Targeting the Altered Pathwaysmentioning

confidence: 99%

Novel Molecular Mechanisms Involved in the Medical Treatment of Pulmonary Arterial Hypertension

Miguel

Cruz‐Utrilla

Oliver

et al. 2023

IJMS

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Pulmonary arterial hypertension (PAH) is a severe condition with a high mortality rate despite advances in diagnostic and therapeutic strategies. In recent years, significant scientific progress has been made in the understanding of the underlying pathobiological mechanisms. Since current available treatments mainly target pulmonary vasodilation, but lack an effect on the pathological changes that develop in the pulmonary vasculature, there is need to develop novel therapeutic compounds aimed at antagonizing the pulmonary vascular remodeling. This review presents the main molecular mechanisms involved in the pathobiology of PAH, discusses the new molecular compounds currently being developed for the medical treatment of PAH and assesses their potential future role in the therapeutic algorithms of PAH.

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“…Adhesion tests to detect platelet adhesion defects in terms of HPS were studied, and all of them were positive. Macitentan was considered in this patient because of its good safety profile and efficacy [3]. After the medical treatment, her 6-minute walk test, which was 175 meters at the beginning, improved to 210 meters at 1 month and 280 meters at 3 months.…”

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confidence: 98%