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Can Combination Therapy with Endothelin Receptor Antagonist and PDE5 Inhibitors Prevent Echocardiographic Findings Suspicious for Pulmonary Arterial Hypertension? Description of a Real-Life Case Series.
Diagnostics (Basel)
July 2024
Department of Clinical and Experimental Medicine, Division of Rheumatology, University of Florence, 50134 Florence, Italy.
Objective: To retrospectively evaluate the incidence rate (IR) of elevated echocardiographic estimated systolic pulmonary artery pressure (sPAP), suspected for pulmonary hypertension (PH), in systemic sclerosis (SSc) patients after the introduction of a combination therapy with bosentan and sildenafil for treatment or prevention of digital ulcers.
Methods: Patients attending the Scleroderma Unit of the Universital Hospital of Careggi from July 2010 to July 2023 were enrolled. Patients older than 18 years old with a history of digital ulcers, treated with bosentan and sildenafil in combination for at least 12 months, were included.
Raynaud's Phenomenon: A Current Update on Pathogenesis, Diagnostic Workup, and Treatment.
Vasc Specialist Int
July 2024
Division of Rheumatology, Department of Internal Medicine, School of Medicine, Kyungpook National University, Daegu, Korea.
Raynaud's phenomenon (RP) is a condition characterized by episodic, excessive vasoconstriction in the fingers and toes, triggered by cold or stress. This leads to a distinctive sequence of color changes in the digits. Pallor indicates reduced blood flow due to oxygen deprivation, while erythema appears as reperfusion.
View Article and Find Full Text PDFArticle Synopsis
- A task force of healthcare professionals created evidence-based recommendations for managing Raynaud's phenomenon and digital ulcers in patients with systemic sclerosis and other connective tissue diseases.
- The recommendations include 5 main principles and 13 specific suggestions, with a focus on coordinated team care and shared decision-making between patients and healthcare providers.
- Nifedipine is recommended as the first-line treatment, with other medications like sildenafil and tadalafil as second-line options, while non-drug interventions are advised but lack strong evidence.
Bosentan effect on echocardiographic systolic pulmonary arterial pressure in systemic sclerosis-related pulmonary hypertension: a systematic review and metanalysis.
Clin Exp Rheumatol
August 2024
Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, Università Campus Bio-Medico, Rome, and Immunorheumatology Unit, Fondazione Policlinico Universitario Campus Bio Medico, Rome, Italy.
Objectives: Bosentan is a dual endothelin receptor antagonist approved for the treatment of SSc digital ulcers (DU) and pulmonary arterial hypertension (PAH). Systolic pulmonary arterial pressure (sPAP) is a relevant parameter for the follow-up and prognosis of SSc-PAH. The therapeutic magnitude of bosentan in SSc-PAH is not fully understood, thus we aim to establish the degree of sPAP reduction in bosentan treated SSc-PAH patients.
View Article and Find Full Text PDFSystematic literature review to inform the Portuguese recommendations for the management of Raynaud's phenomenon and digital ulcers in systemic sclerosis and other connective tissue diseases.
ARP Rheumatol
July 2024
Universidade Nova de Lisboa, Lisbon, Portugal.
Objective: To perform a systematic literature review (SLR) aimed at evaluating the efficacy and safety of pharmacological and non-pharmacological treatments for Raynaud's phenomenon (RP) and digital ulcers (DU) in patients with systemic sclerosis (SSc) and other connective tissue diseases (CTD), in order to inform the Portuguese recommendations for managing RP and DU in these patients.
Methods: A SLR was conducted until May 2022 to identify studies assessing the efficacy and safety of pharmacological and non-pharmacological interventions for RP and DU in SSc and other CTD. Eligible study designs included randomized controlled trials (RCTs), controlled clinical trials, and their extensions for assessing efficacy and safety of interventions.
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