bosentan / Generic mfg. - LARVOL DELTA

A KCa 2.2/2.3 Opener Reverses ET-1-Induced NLRP3 Activation in Hypertensive Mice Corpora Cavernosa. (PubMed, Biomolecules) - "Treatment with the endothelin receptor antagonist bosentan or the KCa 2.2/2.3 channel opener NS13001 reversed these dysfunctions and reduced ET-1-induced NLRP3 activation. NS13001 also restored decreased currents in endothelial cells exposed to ET-1. These findings establish that hypertension-induced erectile dysfunction involves an ET-1/membrane depolarization/NLRP3 inflammasome axis in corpus cavernosum endothelial cells, and that targeting endothelial KCa 2.2/2.3 channels represents a promising therapeutic strategy to counteract erectile dysfunction."

Journal • Preclinical • Cardiovascular • Erectile Dysfunction • Hypertension • Pulmonary Arterial Hypertension • CASP1 • EDN1 • IL1B • NLRP3

Sensitive voltammetric detection of bosentan using gold nanoparticles-decorated pencil graphite electrode in pharmaceutical formulations and plasma samples. (PubMed, Sci Rep) - No abstract available

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Disproportionality analysis of adverse events associated with endothelin receptor antagonists based on the FDA adverse event reporting system (FAERS). (PubMed, Front Cardiovasc Med) - "Endothelin receptor antagonists (ERAs), including bosentan, ambrisentan, and macitentan, are recognized as first-line treatments for pulmonary arterial hypertension (PAH). These results provide valuable insights for clinicians aiming to optimize ERA utilization, minimize associated risks, and improve patient outcomes. Future research should focus on elucidating the underlying mechanisms of these ADEs to further enhance the safety and efficacy of ERA therapies."

Adverse events • Journal • Cardiovascular • Gout • Hematological Disorders • Hypertension • Hypotension • Inflammatory Arthritis • Pain • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Rheumatology • Thrombosis

Retinal Assessment and the Retina-Choroid Link in Precapillary Pulmonary Hypertension: A Single-Center Cross-Sectional Study. (PubMed, Clin Ophthalmol) - "In univariate regression analysis, ORL thickness was significantly associated with choroidal structural parameters (p ≤ 0.007) and bosentan treatment (p = 0.019)...ORL thickness may represent an adjunct structural indicator in PPH. These findings are exploratory and require validation in larger, longitudinal cohorts."

Journal • Observational data • Cardiovascular • Diabetic Retinopathy • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Retinal Disorders

L-carnosine as a biocompatible dipeptide carrier for oral delivery of amorphous bosentan: the binary systems. (PubMed, Int J Pharm) - "The outcomes of two stage biorelevant dissolution showed that both single- and two-phase solid dispersions created supersaturated solutions, but the supersaturation level of the single phase coamorphous formulation exceeded the others with no drug precipitation at the intestinal stage. These findings were further supported by the results of in vitro permeability studies in Caco-2 cell monolayers where the apparent permeability of bosentan from the single phase co-amorphous system loaded with L-carnosine was of 60 % higher than that of the drug alone."

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Human blood vessel organoids recapitulate key mechanisms of transition from vasculopathy to fibrosis in systemic sclerosis. (PubMed, Ann Rheum Dis) - "This study establishes BVOs as a complex human model of SSc vasculopathy and demonstrates in a multiomic approach that they recapitulate disease-specific vascular dysfunction and its transition to fibrosis. We show that genetic susceptibility and pathogenic autoantibodies synergise in driving microvascular injury in SSc. Furthermore, we provide evidence that SSc BVOs are a promising platform for evaluating therapies that prevent the transition from vasculopathy to fibrosis, and present Notch/γ-secretase inhibition as a potential novel target in SSc vasculopathy."

Journal • Fibrosis • Immunology • Scleroderma • Systemic Sclerosis

REFRACTORY DIGITAL ULCERS AND CHRONIC OSTEOMYELITIS COMPLICATING DIFFUSE SYSTEMIC SCLEROSIS: A REAL-LIFE CASE (SSWC 2026) - "Digital ulcers were refractory to nifedipine 60 mg/day, prompting escalation to alprostadil in 2013 and iloprost in 2016, both initially effective...Sildenafil (up to 150 mg/day, 2024–2025) proved ineffective. Bosentan, initiated in January 2025, was discontinued after re-hospitalization in August 2025 for multiple ulcers and chronic osteomyelitis of the right fifth digit...Osteomyelitis developed in the right second digit in 2019, requiring multiple antibiotic courses (flucloxacillin, amoxicillin–clavula- nate, clindamycin, trimethoprim–sulfamethoxazole) following Staphylococcus aureus isolation, with relapse in 2020 managed with ciprofloxacin...Mycophenolate mofetil (2017–2019) was used for progressive skin fibrosis and NSIP but discontinued due to recurrent osteomyeli- tis. Nintedanib (2021–2024) was initiated for progressive pulmonary fibrosis but withdrawn due to severe diarrhea and weight loss... This case illustrates the complex therapeutic challenges in SSc..."

Clinical • Cardiovascular • Fibrosis • Hepatology • Hypotension • Immunology • Infectious Disease • Inflammation • Interstitial Lung Disease • Musculoskeletal Diseases • Orthopedics • Pneumonia • Primary Biliary Cholangitis • Pulmonary Disease • Respiratory Diseases • Scleroderma • Systemic Sclerosis • Venous Ulcer

Outcome of Pediatric Pulmonary Hypertension Patients Requiring ECMO (SCCM 2026) - "100% (31/31) of patients received at least one PAH therapy prior to and during ECMO [31/31 (100%) inhaled nitric oxide or inhaled epoprostenol; 26/31 (84%) sildenafil, tadalafil or riociugat; 18/31 (58%) macitentan, ambrisentan or bosentan; 8/31(26%) selexipag; 10/31(32%) treprostinil. Pediatric PAH is a complex disease due to varied etiologies often requiring multiple medications to effectively manage. Certain subpopulations such as those with single ventricle CHD have higher mortality especially when requiring ECMO. Further study is necessary to determine if earlier identification and management of PAH patients prior to ECMO can improve survival and decrease need for mechanical circulatory support in this high-risk population."

Clinical • Cardiovascular • Heart Failure • Hematological Disorders • Infectious Disease • Pediatrics • Pneumonia • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Respiratory Diseases

Comparative Effectiveness of Endothelin Receptor Antagonists and PDE-5 Inhibitors in PAH (SCCM 2026) - "Adults carrying an ICD-10 code for PAH (I27.0) who started either ERA monotherapy (bosentan, ambrisentan, macitentan) or PDE5I monotherapy (sildenafil, tadalafil) with no prior prostacyclin or combination therapy were eligible. n this large multicentre, propensity-matched analysis of treatment-naive PAH, ERAs and PDE5Is produced indistinguishable 12-month survival, cardiovascular outcomes, and adverse-event rates. These findings support current guidelines that either class is an acceptable first-line option; choice may therefore be guided by individual comorbidity profiles, side-effect tolerance, and cost rather than expectations of differential efficacy."

HEOR • Cardiovascular • Congestive Heart Failure • Heart Failure • Myocardial Infarction • Pulmonary Arterial Hypertension • Respiratory Diseases

MANAGING PREGNANCY IN A PATIENT WITH UNREPAIRED TRUNCUS ARTERIOSUS AND PULMONARY HYPERTENSION (ACC 2026) - "She was on sildenafil, bosentan, and full-dose enoxaparin for thromboembolism prevention...Decision-Making: As pregnancy continued, sildenafil was continued, bosentan stopped, and oxygen, iloprost, and aspirin added... Pregnancy is contraindicated in pulmonary arterial hypertension due to Truncus Arteriosus. The European and American Society of Cardiology advise termination of pregnancy during the first trimester. If continued, management should focus on O₂ >90% and preventing thrombotic events."

Clinical • Cardiovascular • Heart Failure • Hematological Disorders • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

CARDIOPULMONARY REHABILITATION IN SEVERE CONGENITAL HEART DISEASE: INSIGHTS FROM A YOUNG PATIENT WITH EISENMENGER SYNDROME (ACC 2026) - "Case: A 27-year-old male with perimembranous ventricular septal defect (VSD) and Eisenmenger physiology, on triple pulmonary vasodilator therapy (sildenafil, bosentan, and iloprost), presented with New York Heart Association (NYHA) class II/III dyspnea. Despite significant ventilatory and perfusion inefficiencies, structured rehabilitation improved key prognostic markers. This case highlights the feasibility and clinical benefit of exercise training in adults with Eisenmenger syndrome."

Clinical • Cardiovascular • Heart Failure • Pulmonary Disease

PULMONARY VASODILATOR THERAPY IN SCHISTOSOMIASIS-ASSOCIATED PULMONARY ARTERIAL HYPERTENSION: DEMONSTRATED BENEFIT AND NEED FOR FURTHER STUDIES. (ACC 2026) - "He improved with intravenous furosemide and was discharged on bosentan, tadalafil, and torsemide. Sch-PAH represents a delayed yet clinically significant sequela of bilharziasis. Our case illustrates the benefit of targeted PAH therapy in this population and emphasizes the need for prospective studies and registries in endemic regions."

Cardiovascular • Hepatology • Infectious Disease • Pulmonary Arterial Hypertension • Respiratory Diseases

AN AUDIT OF ILOPROST DELIVERY IN A TERTIARY SCLERODERMA SPECIALIST CENTRE (SSWC 2026) - "This included Iloprost indi- cation, frequency/duration of infusion and concomitant oral vasodilator therapy (including calcium channel antagonists, angiotensin receptor blockers, phosphodiesterase-5 inhibitors and bosentan). This data aligns with the 2024 BSR SSc guideline recommendation of the need for a dedicated day-case unit for Iloprost administration for a specific SSc patient cohort in addition to a severe PR/EM cohort. Optimisation of the Iloprost delivery pathway has potential to enhance better clinical outcomes and ensure more cost-efficiency."

Cardiovascular • Immunology • Scleroderma • Systemic Sclerosis

A MICROCOSTING STUDY OF CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION (CTEPH) BASED ON REAL WORLD DATA FROM BRAZIL (ISPOR 2026) - "OBJECTIVES: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease that has pulmonary endarterectomy (PEA) as the gold standard treatment, while ballon pulmonary angioplasty (BPA) and guanylate cyclase stimulator (riociguat) are recommended for patients who cannot undergo surgery or who relapse after PEA. Patients with CTEPH account for an excessive burden on the system. Strategies to reduce the impact of the disease should be pursued from both clinical and financial perspectives."

Clinical • Real-world • Real-world evidence • Cardiovascular • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Rare Diseases • Respiratory Diseases

ASSESSMENT OF QUALITY OF LIFE IN PATIENTS WITH CHRONIC KIDNEY DISEASE AND PULMONARY HYPERTENSION (NKF-SCM 2026) - "CONCLUSION Six months of bosentan therapy in CKD stages 3a-4 yielded clinically meaningful improvements in health-related quality of life (CAMPHOR scores decreased by 3.2-5.5 points) while sPAP remained unchanged and TAPSE showed modest, stage-specific changes. These results suggest that symptomatic and functional gains can occur independently of short-term reductions in pulmonary pressures, supporting routine use of CAMPHOR alongside echocardiography to monitor treatment benefit in this population."

Clinical • HEOR • Cardiovascular • Chronic Kidney Disease • Nephrology • Pulmonary Arterial Hypertension • Pulmonary Disease • Renal Disease • Respiratory Diseases

A Rare Movement Disorder in Systemic Lupus Erythematosus and Antiphospholipid Syndrome: Chorea/Ballismus (LUPUS 2026) - "Current medications included mycophenolate mofetil, methylprednisolone, nifedipine, bosentan, and aspirin, along with intermittent iloprost and hyperbaric oxygen therapy for digital ulcers. The patient's recurrent anticardiolipin IgM positivity supports a thrombotic mechanism, while the favorable response to anticoagulation and rituximab highlights the dual need to address both vascular and inflammatory pathways. Recognition of such cases is critical for early intervention, as combined immunomodulatory and antithrombotic therapy can lead to substantial neurological recovery."

Immunology • Inflammatory Arthritis • Lupus • Systemic Lupus Erythematosus

Impact of Switching From Ambrisentan to Bosentan on Clinical and Risk Profiles in Pulmonary Arterial Hypertension: Insights From the Action Study (ATS 2026) - No abstract available

Clinical • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

HUMAN BLOOD VESSEL ORGANOIDS AS A MODEL OF VASCULOPATHY IN SSC (SSWC 2026) - "Bosentan (BST) and the γ-secretase inhibitor DAPT were tested for their effects in preventing angiogenesis defects in BVOs... We show that SSc BVOs recapitulate key features of SSc vasculopathy, including impaired angiogenesis and enhanced EndMT, increases in pathologic endothelial cell populations, as well as loss of endothelial cells-pericytes interactions when exposed to SSc serum. We further validate that BVOs can be used for drug testing in SSc, and employ them to demonstrate that inhibition of Notch signaling might be a novel approach for targeting vasculopathy in SSc."

Systemic Sclerosis

EVALUATION OF SKIN INVOLVEMENT AND DISEASE ACTIVITY IN GREEK PATIENTS WITH SYSTEMIC SCLEROSIS TREATED WITH BOSENTAN DURING A 18-MONTH FOLLOW-UP PERIOD: RESULTS FROM THE CURE CLINICAL STUDY (SSWC 2026) - P=N/A | "Univariate analysis showed that patients receiving prostanoids within 6 months prior to baseline as well as those treated with cyclophosphamide during the observation period, had a greater reduction in MRSS at last visit (p=0.001 and p=0.011, respectively). The CURE observational study showed an improvement in both MRSS and EUSTAR activity index in Greek patients with SSc treated with bosentan over 18 months."

Clinical • Immunology • Inflammation • Scleroderma • Systemic Sclerosis

HUMAN BLOOD VESSEL ORGANOIDS AS A MODEL OF VASCULOPATHY IN SSC (SSWC 2026) - "Bosentan (BST) and the γ-secretase inhibitor DAPT were tested for their effects in preventing angiogenesis defects in BVOs... We show that SSc BVOs recapitulate key features of SSc vasculopathy, including impaired angiogenesis and enhanced EndMT, increases in pathologic endothelial cell populations, as well as loss of endothelial cells-pericytes interactions when exposed to SSc serum. We further validate that BVOs can be used for drug testing in SSc, and employ them to demonstrate that inhibition of Notch signaling might be a novel approach for targeting vasculopathy in SSc."

Systemic Sclerosis

IMPACT OF IMMUNOSUPPRESSIVE THERAPY ON BURDEN OF RECURRENT DIGITAL ULCERS IN SCLERODERMA VASCULOPATHY (SSWC 2026) - "Bosentan, a dual oral endothelin receptor antagonist treatment was associated with a 30% reduction in the number of new DUs compared with placebo... In this retrospective single-centre study with severe digital vasculopathy, the concurrent use of immunosuppressive therapy was not associated with reduced ulcer recurrence. Importantly, there was no increased risk of infection observed in the immunosuppressive cohort. Larger, multicentre prospective studies are warranted to confirm these observations and better inform clinical decision-making."

Immunology • Infectious Disease • Inflammation • Scleroderma • Systemic Sclerosis

REAL-LIFE OBSERVATIONAL STUDY TO EVALUATE RAYNAUD'S SEVERITY AND THE CHANGE IN DIGITAL ULCERS IN GREEK PATIENTS WITH SYSTEMIC SCLEROSIS TREATED WITH BOSENTAN. THE CURE STUDY (SSWC 2026) - P=N/A | "The CURE observational study showed a significant improvement in the severity of Raynaud's phenomenon with a parallel reduction in the number of DUs in Greek patients with SSc treated with bosentan for 18 months."

Clinical • Observational data • Cardiovascular • Immunology • Pulmonary Disease • Respiratory Diseases • Scleroderma • Systemic Sclerosis

ASSOCIATION BETWEEN VASOACTIVE-VASODILATING THERAPY AND REDUCED DETECTION OF PULMONARY ARTERIAL HYPERTENSION IN SYSTEMIC SCLEROSIS: EVIDENCE FROM A EUSTAR STUDY (SSWC 2026) - "Our data show that bosentan is associated with less frequent diagnosis of PAH and precapillary PH, in particular when employed in patients with current DU at the time of RHC. Our results support further research to optimize timing and patient selection for VVD therapy in SSc, aiming also at additional preventive effects at pulmonary level."

Cardiovascular • Immunology • Pulmonary Arterial Hypertension • Respiratory Diseases • Scleroderma • Systemic Sclerosis

Endothelin-1 in the failing Fontan: pathobiology, precision therapeutics, and future trial design. (PubMed, Front Pediatr) - "Trials of endothelin-receptor antagonists (bosentan, ambrisentan, macitentan) demonstrate reassuring safety and suggest benefit when outcomes emphasize ventilatory efficiency or hepatic endpoints rather than peak oxygen consumption, which is physiologically constrained in Fontan physiology. Given the mixed results of existing trials, a framework is outlined that stratifies Fontan patients into pulmonary-inefficiency, congestive-hepatic, lymphatic, and arrhythmia-dominant phenotypes, using co-primary endpoints such as VE/VCO2 slope, elastography, and biomarker panels. By linking ET-1 biology to pragmatic trial design, this approach emphasizes targeted strategies that may stabilize the circulation, extend transplant candidacy, and improve long-term outcomes."

Journal • Review • Cardiovascular • Heart Failure • Hepatology • Hypertension • Transplantation • EDN1 • RHOA

Silver nanoparticles-based green fluorescent probe for determination of Bosentan in pharmaceutical formulation and spiked plasma samples. (PubMed, BMC Chem) - "Over the concentration range of 0.02 to 0.12 µg/mL, the quenching impact of BOS on AgNPs increased as its concentration increased. The suggested method's greenness was assessed using two metric systems: the Analytical Eco-scale (AES) with a score of 80 and the AGREE greenness assessment tool with a score of 0.68."

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