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Systemic Sclerosis: From Pathophysiology to Novel Therapeutic Approaches.

Systemic sclerosis (SSc) is a systemic, immune-mediated chronic disorder characterized by small vessel alterations and progressive fibrosis of the skin and internal organs. The combination of a predisposing genetic background and triggering factors that causes a persistent activation of immune system at microvascular and tissue level is thought to be the pathogenetic driver of SSc. Endothelial alterations with subsequent myofibroblast activation, excessive extracellular matrix (ECM) deposition, and unrestrained tissue fibrosis are the pathogenetic steps responsible for the clinical manifestati

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… Bosentan, ambrisentan, and macitentan are the endothelin receptor antagonists currently approved for the treatment of PAH, including connective tissue disease associated-PAH. … Bosentan in the treatment of pulmonary arterial hypertension with the focus on the mildly symptomatic patient. Vasc. Health Risk Manag. 2009;5:607–619. doi: 10.2147/VHRM.S4713. 43. …

Race and sex differences in response to endothelin receptor antagonists for pulmonary arterial hypertension.

BACKGROUND: Recently studied therapies for pulmonary arterial hypertension (PAH) have improved outcomes among populations of patients, but little is known about which patients are most likely to respond to specific treatments. Differences in endothelin-1 biology between sexes and between whites and blacks may lead to differences in patients' responses to treatment with endothelin receptor antagonists (ERAs). METHODS: We conducted pooled analyses of deidentified, patient-level data from six randomized placebo-controlled trials of ERAs submitted to the US Food and Drug Administration to elucida

Prevalence of spontaneous portosystemic shunts in patients with portopulmonary hypertension and effect on treatment.

BACKGROUND & AIMS: We documented the frequency of large spontaneous portosystemic shunts in patients with moderate or severe portopulmonary hypertension (POPH) and determined the association between large shunts and response to treatment. METHODS: We performed a retrospective case-control study of data from patients with mild (mean pulmonary artery pressure [MPAP], 25-35 mm Hg; n = 18), moderate (MPAP, 35-50 mm Hg; n = 45), and severe POPH (MPAP, >50 mm Hg; n = 16). Data were compared with those from controls (normal echocardiography with estimated right ventricular systolic pressure, <35 mm

Novel therapeutic approaches for pulmonary fibrosis.

Pulmonary fibrosis represents the end stage of a number of heterogeneous conditions and is, to a greater or lesser degree, the hallmark of the interstitial lung diseases. It is characterized by the excessive deposition of extracellular matrix proteins within the pulmonary interstitium leading to the obliteration of functional alveolar units and in many cases, respiratory failure. While a small number of interstitial lung diseases have known aetiologies, most are idiopathic in nature, and of these, idiopathic pulmonary fibrosis is the most common and carries with it an appalling prognosis - med

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… trend in delayed time to disease progression or death in the bosentan arm of IPF patients who had undergone lung biopsy | | BUILD-3 (Actelion, Switzerland) | Prospective, double-blinded, randomized placebo-controlled trial; total n = 616, bosentan : placebo 2:1 recruitment complete; | Primary end points … Although no significant difference between the bosentan and placebo arms was observed in the primary end point of 6 minute walk test distance, a trend in favour of bosentan was observed in the secondary end point of time to death or disease progression. …

Systemic Sclerosis Associated Interstitial Lung Disease: New Directions in Disease Management.

A subgroup of patients with systemic sclerosis (SSc) develop interstitial lung disease (ILD), characterized by inflammation and progressive scarring of the lungs that can lead to respiratory failure. Although ILD remains the major cause of death in these individuals, there is no consensus statement regarding the classification and characterization of SSc-related ILD (SSc-ILD). Recent clinical trials address the treatment of SSc-ILD and the results may lead to new disease-altering therapies. In this review, we provide an update to the diagnosis, management and treatment of SSc-ILD.

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… Bosentan Bosentan, is a nonselective endothelin receptor antagonist, used in the treatment of pulmonary hypertension. It is known that the endothelin system participates in the pathogenesis of SSc, and that it could delay the progression of SSc-ILD. … Randomized, prospective, placebo-controlled trial of bosentan in interstitial lung disease secondary to systemic sclerosis . Arthritis Rheumatol . ( 2010 ) 62 : 2101 – 8 . 10.1002/art.27466 20506355 67. Daoussis D Liossis SN Tsamandas AC Kalogeropoulou C Paliogianni F Sirinian C . …

A comparative analysis of transcriptomics of newly diagnosed multiple myeloma: exploring drug repurposing.

Multiple myeloma (MM) is an incurable malignant plasma cell disorder characterized by the infiltration of clonal plasma cells in the bone marrow compartment. Gene Expression Profiling (GEP) has emerged as a powerful investigation tool in modern myeloma research enabling the dissection of the molecular background of MM and allowing the identification of gene products that could potentially serve as targets for therapeutic intervention. In this study we investigated shared transcriptomic abnormalities across newly diagnosed multiple myeloma (NDMM) patient cohorts. In total, publicly available tr

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… Pharmaceutical EDNR blockage in MM cell lines with bosentan or macitentan decreased viability of cultured cells ( 88 , 89 ). … Similarly, combination of bosentan and bortezomib had stronger antiproliferative effects in myeloma cell lines than bosentan or bortezomib alone implying a synergistic effect of EDNR antagonists and proteasome inhibitors ( 87 ). …

Системный склероз-ассоциированная легочная гипертензия: спектр и воздействие

Систематическая склерозная гипертония легких (SSc-PAH) является катастрофическим осложнением one из наиболее распространенных и разрушительных аутоиммунных заболеваний. После диагностирования он становится главной причиной смертности среди этой пациентки. Различные организации и общества разработали и подтвердили методы обследования и оценки рисков, с тем чтобы выявить пациентов на раннем этапе их курса лечения и незамедлительно направить их в экспертные центры для гемодинамической оценки и официальной диагностики. Кроме того, в настоящее время в ряде крупных многоцентровых клинических испытан

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… Endothelin Receptor Antagonists Bosentan, ambrisentan, and macitentan are ERAs approved for the treatment of SSc-PAH. … Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases. Ann. Rheum. Dis. 2007;67:1222–1228. doi: 10.1136/ard.2007.079921. 90. …

Successful treatment of pulmonary hypertension following hematopoietic stem cell transplant with a single oral tadalafil: a case report.

A 46-year-old man who had undergone hematopoietic stem cell transplant twice because of acute lymphoblastic leukemia with recurrence presented with dyspnea, leading to a diagnosis of pulmonary arterial hypertension which was quickly and effectively treated with the phosphodiesterase type 5 inhibitor tadalafil. To our knowledge, pulmonary arterial hypertension related to hematologic malignancies requiring hematopoietic stem cell transplant is rarely reported. Importantly, the present case suggests that early diagnosis and treatment with a pulmonary vasodilator, such as tadalafil, can greatly de

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… . | 36 | F | CML | Hydroxyurea | BU, CY | 12.5 mo | CT, TEE, PFT, open lung bx, | Bosentan, Steroid | Alive at 29 mo Limsuwan et al. | 20 | F | CML | Imatinib | AraC, CY, TBI | 4 mo | CT, TTE, RHC | Sildenafil, Beraprost | Alive at 30 mo Nakaoka et al. | 35 | F | AML | Unknown | BU, CY | Day 94 | TTE … Compared to other reported pulmonary vasodilator regiments ( Table 2 ), tadalafil monotherapy is generally well tolerated without need for monthly liver function test (LFT) monitoring (as in the case of bosentan), reduced side effect profiles compared to prostacyclin analogues (as in the case of beraprost …

Талидомид взаимодействует с воспалением в идиопатическом фиброзе легких.

&quot; Трагедия Талидомида &quot; является важной вехой в истории фармацевтической промышленности. Несмотря на ограниченные клинические испытания, прилагаются постоянные усилия по изучению Талидомида в качестве лекарства от рака и подстрекательских заболеваний, таких, как ревматуидный артрит, проказа, и COVID-19. В рамках этого обзора основное внимание уделяется возможностям борьбы с воспалением путем повторного использования талидомида для лечения идиопатического фиброза легких (ИПФ). Статьи были найдены в базе данных Scopus, сортированы и рассмотрены отдельные статьи. Содержание включает в с

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… A prospective, randomized, double-blind, placebo-controlled, parallel-group, event-driven, morbidity–mortality clinical trial of bosentan, (an endothelin receptor antagonist) was conducted on 616 patients to investigate the benefit of modulating pulmonary hypertension associated with IPF (Giordano et … However, bosentan failed to show any efficacy because there were no differences between treatment groups in health-related quality of life or dyspnoea (King et al. 2011 ). …

Therapeutic targets for bone metastases in breast cancer.

Breast cancer is prone to metastasize to bone. Once metastatic cells are in the bone marrow, they do not, on their own, destroy bone. Instead, they alter the functions of bone-resorbing (osteoclasts) and bone-forming cells (osteoblasts), resulting in skeletal complications that cause pathological fractures and pain. In this review, we describe promising molecular bone-targeted therapies that have arisen from recent advances in our understanding of the pathogenesis of breast cancer bone metastases. These therapies target osteoclasts (receptor activator of nuclear factor kB ligand, integrin αvβ3

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… The mixed (ET A and ET B ) inhibitor bosentan also inhibits murine 4T1 breast cancer bone metastases in vivo [ 41 ]. Furthermore, ET-1 and ET-2 increase breast cancer cell migration and invasion in vitro [ 42 ]. … Bosentan inhibits tumor vascularization and bone metastasis in an immunocompetent skin-fold chamber model of breast carcinoma cell metastasis. Clin Exp Metastasis. 2006;23:41–53. doi: 10.1007/s10585-006-9016-z. Grimshaw MJ, Hagemann T, Ayhan A, Gillett CE, Binder C, Balkwill FR. …

Autoantibodies as Biomarker and Therapeutic Target in Systemic Sclerosis.

Systemic sclerosis (SSc) is a rare connective tissue disorder characterized by immune dysregulation evoking the pathophysiological triad of inflammation, fibrosis and vasculopathy. In SSc, several alterations in the B-cell compartment have been described, leading to polyclonal B-cell hyperreactivity, hypergammaglobulinemia and autoantibody production. Autoreactive B cells and autoantibodies promote and maintain pathologic mechanisms. In addition, autoantibodies in SSc are important biomarkers for predicting clinical phenotype and disease progression. Autoreactive B cells and autoantibodies rep

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… Moreover, bosentan showed beneficial effects in the treatment of Raynaud’s phenomenon and SSc-related digital ulcers [ 145 , 146 , 147 , 148 ]. In the majority of SSc patients, anti-AT1R and anti-ETAR autoantibodies are detectable. …

Обзор различных методов совершенствования лечения пульмональной гипертензии у пациентов с системным склерозом

При системном склерозе (SSc) смертность связана главным образом с участием лёгких, для которых характерны межститиальная болезнь легких (МЛС) и легочная гипертония (PH). В SSc PH может быть вызван различными этиологиями, включая ILD, хроническую тромбоэмболическую болезнь, легочную веноклюзивную болезнь и легочную артериальную гипертонию (PAH). Основным инструментом для экранирования ПАУ является трансхуракическая эхокардиография (TTE), которая обладает чувствительностью: 90%, даже если окончательный диагноз должен быть подтвержден правильной катетеризацией сердца (RHC). Радиологическая оценка

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… Bosentan (BREATH-1 trial), macitentan (SERAPHIN trial) and ambrisentan (ARIES 1 and 2 trials) are endothelin receptor-antagonists which are approved for the treatment of SSc-PAH [ 76 , 77 , 78 , 79 , 80 ]. 4.4.4. … Bosentan Therapy for Pulmonary Arterial Hypertension N. Engl. J. Med. 2002 346 896 903 10.1056/NEJMoa012212 11907289 77. Galiè N. Olschewski H. Oudiz R.J. Torres F. Frost A. Ghofrani A. Badesch D.B. McGoon M.D. McLaughlin V.V. Roecker E.B. …

A rare case of POEMS syndrome presenting as essential thrombocythemia.

Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes (POEMS) is a rare paraneoplastic syndrome, and its diagnosis is based on a series of clinical and laboratory findings. We present the case of a 46-year-old woman who was previously diagnosed with essential thrombocythemia. The patient complained about dyspnea on exertion, nausea, burning of the lower limbs, weight loss, recurrent episodes of lower back pain and polymenorrhea. Physical examination revealed hyperpigmentation, livedo reticularis of the lower limbs, sclerodermoid changes and plectrodactyly. A computed t

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… Additional symptomatic treatment included furosemide, nifedipine and bosentan. Six months later, the patient showed a complete hematologic response and a partial radiologic response on a fluorodeoxyglucose positron emission tomography scan. …

Bisphosphonates for the Treatment of Calcinosis Cutis-A Retrospective Single-Center Study.

(1) Background: Calcinosis cutis is a frequent symptom of autoimmune connective tissue diseases leading to pain, transcutaneous expulsion of calcified material and bacterial superinfection. There is a high need for new therapeutic options as no standardized treatment algorithm is established. While case reports indicate beneficial effects of bisphosphonates, standardized evaluation of treatment effects is missing. (2) Methods: In this retrospective analysis we evaluate the effects of intravenous pamidronate, a second-generation bisphosphonate, in seven patients with calcinosis cutis using cons

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… 75 | 75 previous treatments | intravenous immuno-globulins cyclophosphamid methyl-prednisolone azathioprine | methotrexate prednisolone azathioprine | intravenous immuno-globuline methotrexate prednisolone azathioprine chloroquine PUVA, iloprost | methotrexate prednisolone cyclosporine chloroquine | bosentan …

Keyword Index for Volume 88

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… bladder cancer 92, 586, 1492, 1932 bleomycin 1764 blood supply 291 blood vessel invasion 1894 BMI 36 BNIP3L 270 body mass 1038 body mass index 1388 body weight 679 bone infiltration 1105 bone marrow 855 bone metastasis 195, 1318 bone resorption 1318 bone scan 195 bone sialoprotein 396 bosentan …

A Rapid Review of Adenocarcinoma and Pulmonary Tumor Thrombotic Microangiopathy: A Deadly Duo.

Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare paraneoplastic syndrome associated with various adenocarcinomas, most commonly gastric adenocarcinoma. This condition can progressively worsen pulmonary arterial hypertension, leading to acute or subacute pulmonary heart failure and respiratory insufficiency. This paper examines the pathogenesis, clinical presentation, diagnosis, treatment, and prognosis of PTTM. Given PTTM's poor prognosis, we emphasize treatment strategies. PTTM in adenocarcinoma patients can mimic other pulmonary diseases, causing diagnostic delays. Current PTTM tr

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… Apart from oxygen therapy, the medications used to treat PTTM include those for advanced PAH, such as sildenafil, tadalafil, ambrisentan, bosentan, and epoprostenol [ 62 ]. …

Potential impact of the implementation of multiple-criteria decision analysis (MCDA) on the Polish pricing and reimbursement process of orphan drugs.

BackgroundThe objective of this study was to assess the potential impact of the implementation of multiple-criteria decision analysis (MCDA) on the Polish pricing and reimbursement (P&R) process with regard to orphan drugs.MethodsA four step approach was designed. Firstly, a systematic literature review was conducted to select the MCDA criteria. Secondly, a database of orphan drugs was established. Thirdly, health technology appraisals (HTA recommendations) were categorized and an MCDA appraisal was conducted. Finally, a comparison of HTA and MCDA outcomes was carried out. An MCDA outcome was

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… treatment | Positive | Restricted with time limits 5 | Somavert (Pegvisomant) | Acromegaly | Negative | Insufficient clinical effectiveness, not cost-effective vs standard of care 6 | Ventavis (Iloprost) | Pulmonary arterial hypertension (PAH) | Positive | Restricted to subpopulations 7 | Tracleer (Bosentan …

Epigenetic modification in diabetic kidney disease.

Diabetic kidney disease (DKD) is a common microangiopathy in diabetic patients and the main cause of death in diabetic patients. The main manifestations of DKD are proteinuria and decreased renal filtration capacity. The glomerular filtration rate and urinary albumin level are two of the most important hallmarks of the progression of DKD. The classical treatment of DKD is controlling blood glucose and blood pressure. However, the commonly used clinical therapeutic strategies and the existing biomarkers only partially slow the progression of DKD and roughly predict disease progression. Therefor

Proceedings of International Conference on Cell Death in Cancer and Toxicology. February 20-22, 2018. Guest Editors: Kausar M. Ansari, CSIR-IITR, India and Dhyan Chandra, Roswell Park, USA.

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… Engraftment of LSEC with inactivation of ETA/B receptors through incubation with bosentan was superior. … This allowed accelerated proliferation in transplanted bosentan treated LSEC during liver repopulation. We discovered bosentan‐treated LSEC were protected from oxidative DNA damage due to lower levels of reactive oxygen species and from double‐strand DNA breaks with preservation of ATM expression. …

SUBJECT INDEX Volume 125 (JANUARY–JUNE, 2004)

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… Breath , 1597 Lung Transplantation , 1968 Management of Cardiac Arrhythmias , 1597 Non-Neoplastic Disorders of the Lower Respiratory Tract: Atlas of Nontumor Pathology , 1176 Noninvasive Mechanical Ventilation , 803 Sleep Medicine Pearls , 357 BOOP: See Bronchiolitis obliterans organizing pneumonia Bosentan … Supp., vol. 124:115S; clarification, vol. 125:356; bosentan in pulmonary artery hypertension secondary to systemic sclerosis, Oct. Supp., vol. 124:223S; clarification, vol. 125:356; significance of allogeneic blood transfusion on decreased survival in patients with esophageal adenocarcinoma, Oct. …