Pharmacotherapy of Systemic Sclerosis: Focus on Bosentan

2009 ◽  
Vol 1 ◽  
pp. 1179559X0900100
Author(s):  
Kamyar Afshar ◽  
Ayana Boyd-King
Keyword(s):  
Quality Of Life ◽  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Medical Management ◽  
Therapeutic Option ◽  
Organ Systems ◽  
Potent Vasoconstrictor ◽  
Clinical Worsening ◽  
Indications For Use

Systemic sclerosis is manifested by the massive deposition of collagen within various organ systems. Endothelin-1 has taken a pivotal role as not only a potent vasoconstrictor, but also as a profibrotic intermediary. Bosentan is proving to be a viable therapeutic option in alleviating manifestations of systemic sclerosis other than pulmonary hypertension. There are many reports in the literature profiling bosentan's safety, efficacy and tolerability in patients. In addition, it has greatly improved patients’ quality of life, reduced the number of hospitalizations and increased the time to clinical worsening of the disease. The clinical indications for use of bosentan in a wide array of patients has served as a valuable asset in the medical management of systemic sclerosis.

scholarly journals Adaptation and validation of the quality of life assessment of the Cambridge pulmonary hypertension outcome review (CAMPHOR) for Brazil

2020 ◽  
Vol 4 (1) ◽  
Author(s):  
Ricardo Amorim Corrêa ◽  
Monica Corso Pereira ◽  
Mariana Ferreira Bizzi ◽  
Rafael W. R. de Oliveira ◽  
Camila Farnese Rezende ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Pulmonary Hypertension ◽  
Life Assessment ◽  
Quality Of Life Assessment

scholarly journals Pulsed Dose Rate Brachytherapy of Lip Carcinoma: Clinical Outcome and Quality of Life Analysis

Cancers ◽  
2021 ◽  
Vol 13 (6) ◽  
pp. 1387
Author(s):  
Radouane El Ayachy ◽  
Roger Sun ◽  
Kanta Ka ◽  
Adrien Laville ◽  
Anne-Sophie Duhamel ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Dose Rate ◽  
Therapeutic Option ◽  
Local Control Rate ◽  
Local Failure ◽  
Life Assessment ◽  
Grade Ii ◽  
Lip Carcinoma ◽  
Pulsed Dose Rate

Purpose: Lip carcinoma represents one of the most common types of head and neck cancer. Brachytherapy is a highly effective therapeutic option for all stages of lip cancers. We report our experience of pulsed dose rate brachytherapy (PDR) as treatment of lip carcinoma. Methods and Materials: this retrospective single center study included all consecutive patients treated for a lip PDR brachytherapy in our institution from 2010 to 2019. The toxicities and outcomes of the patients were reported, and a retrospective quality of life assessment was conducted by phone interviews (FACT H&N). Results: From October 2010 to December 2019, 38 patients were treated in our institution for a lip carcinoma by PDR brachytherapy. The median age was 73, and the majority of patients presented T1-T2 tumors (79%). The median total dose was 70.14 Gy (range: 60–85 Gy). With a mean follow-up of 35.4 months, two patients (5.6%) presented local failure, and seven patients (19%) had lymph node progression. The Kaplan–Meier estimated probability of local failure was 7.2% (95% CI: 0.84–1) at two and four years. All patients encountered radiomucitis grade II or higher. The rate of late toxicities was low: three patients (8.3%) had grade II fibrosis, and one patient had grade II chronic pain. All patients would highly recommend the treatment. The median FACT H&N total score was 127 out of 148, and the median FACT H&N Trial Outcome Index was 84. Conclusions: This study confirms that an excellent local control rate is achieved with PDR brachytherapy as treatment of lip carcinoma, with very limited late side effects and satisfactory functional outcomes. A multimodal approach should help to improve regional control.

scholarly journals ILD-specific health-related quality of life in systemic sclerosis-associated ILD compared with IPF

2020 ◽  
Vol 7 (1) ◽  
pp. e000598
Author(s):  
Michael T Durheim ◽  
Anna-Maria Hoffmann-Vold ◽  
Tomas M Eagan ◽  
Arnt-Ove Hovden ◽  
May Brit Lund ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Function ◽  
Forced Vital Capacity ◽  
Vital Capacity ◽  
Health Related Quality ◽  
Related Quality ◽  
Health Related

IntroductionIdiopathic pulmonary fibrosis (IPF) and systemic sclerosis-associated interstitial lung disease (SSc-ILD) are fibrotic ILDs with divergent disease populations. Little is known about health-related quality of life (HRQL) in SSc-ILD relative to IPF.MethodsWe used the Kings Brief Interstitial Lung Disease Questionnaire (K-BILD) to compare HRQL in a cross-sectional study of 57 patients with IPF and 29 patients with SSc-ILD. Analysis of covariance was used to adjust for age, gender and lung function.ResultsThe unadjusted mean K-BILD score was 63.1 (95% CI 57.1 to 69.1) among patients with SSc-ILD, as compared with 54.7 (51.8–57.5) among those with IPF (p=0.005). However, this difference in HRQL was attenuated after adjustment for age, gender and lung function. In a multivariable model, only forced vital capacity was associated with K-BILD scores. K-BILD scores were correlated with both forced vital capacity and with other relevant HRQL measures, regardless of ILD diagnosis.DiscussionPatients with SSc-ILD may have better ILD-specific quality of life than patients with IPF, but this difference appears to be driven primarily by better lung function. These results underscore the impact of lung function on HRQL in fibrotic ILD and the utility of K-BILD to assess HRQL in SSc-ILD.

scholarly journals Clinical evaluation of pulmonary hypertension using patient-reported outcomes: a cross-sectional study

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Miguel Ángel Amor-García ◽  
Sara Ibáñez-García ◽  
Xandra García-González ◽  
Teresa Mombiela ◽  
Cristina Villanueva-Bueno ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Pulmonary Hypertension ◽  
Clinical Evaluation ◽  
Patient Reported Outcomes ◽  
Specific Treatment ◽  
Tertiary Hospital ◽  
Functional Class ◽  
Cross Sectional ◽  
Patient Reported

Abstract Background Patients with pulmonary hypertension (PH) have progressive and disabling symptoms, as well as a burden of treatments and a difficult clinical evaluation that make health-related quality of life a particularly relevant endpoint in this disease. The objective of the study was to evaluate patient-reported outcomes of patients receiving specific treatment for PH in a tertiary hospital using a specific questionnaire (Cambridge Pulmonary Hypertension Outcome Review-CAMPHOR) in the pharmacy consultation. Methods A cross-sectional, observational, descriptive study was conducted. It included all patients receiving specific treatment for PH in a tertiary hospital in Madrid, Spain. The inclusion period comprised between August to December 2019. CAMPHOR questionnaires containing three domains: symptoms, activities and quality of life were completed by the patients at the pharmacy consultation. Demographic and clinical variables, including WHO Functional Class (WHO FC), PH-specific tests and hemodynamic parameters, were recorded. Non-parametric analyses to assess relations between variables and CAMPHOR domains were performed. Results Thirty-six patients consented to participate in the study and completed the questionnaire. Median scores for symptoms, activities, and quality of life domains were 5.5 (2.5–10), 8.0 (4.5–10.5) and 3.5 (1–7.5), respectively. Statistically significant differences were found in the three domains when comparing by WHO FC, in the activities domain for 6-m walking test and in the quality of life domain for patients who had emergency visits or hospitalizations in the last year. Conclusions The CAMPHOR questionnaire could be useful as a complementary test to achieve an integrated evaluation of PH patients, who could complete it easily during their routine pharmacy visits.

scholarly journals EXPRESS: The benefit of exercise-based rehabilitation programmes in patients with pulmonary hypertension: a systematic review and meta-analysis of randomized controlled trials

2021 ◽  
pp. 204589402110078
Author(s):  
Lu Yan ◽  
Wence Shi ◽  
Zhi-hong Liu ◽  
Qin Luo ◽  
Zhihui Zhao ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Pulmonary Hypertension ◽  
Randomized Controlled Trials ◽  
Exercise Capacity ◽  
Meta Analysis ◽  
Controlled Trials ◽  
Randomized Controlled ◽  
The Mean ◽  
Quality Evidence

Background: Several studies have suggested that exercise capacity and quality of life are reduced in patients with pulmonary hypertension (PH), and exercise-based rehabilitation can improve exercise capacity and quality of life in patients with PH. The aim of this study is to assess the efficacy and safety of exercise-based rehabilitation in patients with PH through a meta-analysis of randomized controlled trials. Methods: We searched PubMed, Embase, Medline, and the Cochrane Central Register of Controlled Trials up to November 2018. All randomized controlled trials (RCTs) comparing exercise capacity and quality of life between patients undergoing exercise-based rehabilitation and those undergoing non-exercise training were included. Data were extracted separately and independently by two investigators, and discrepancies were arbitrated by the third investigator. We used the random-effects model to analyze the results, the GRADE to assess the risk of bias in the included studies, and I ² statistic to estimate the degree of heterogeneity. Results: Nine RCTs are included, however, only seven RCTs were able to extract data. Including inpatients and outpatients, the total number of participants was 234, most of whom were diagnosed as pulmonary artery hypertension (PAH). The study duration ranged from 3 to15 weeks. The mean six-minute walk distance after exercise training was 51.94 metres higher than control (27.65 to 76.23 metres, n=234, 7 RCTs, low quality evidence), the mean peak oxygen uptake  was 2.96 ml/kg/minute higher (2.49 to 3.43 ml/kg/minute, n=179, 4 RCTs, low-quality evidence) than in the control group . Concluded: Our finding suggest that an exercise-based training program positively influences exercise capacity in patients with PH.

scholarly journals FRI0233 IMPACT AND ADHERENCE TO THE MEDITERRANEAN DIET IN SYSTEMIC SCLEROSIS ITALIAN PATIENTS: CORRELATION WITH GASTROINTESTINAL SYMPTOMS, MOOD DISTURBANCES AND QUALITY OF LIFE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 700.2-700
Author(s):  
G. De Luca ◽  
G. Natalello ◽  
G. Abignano ◽  
C. Campochiaro ◽  
D. Temiz Karadağ ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Systemic Sclerosis ◽  
Mediterranean Diet ◽  
Dietary Habits ◽  
Anxiety And Depression ◽  
Research Support ◽  
Work Impairment ◽  
Gi Symptoms ◽  
The Mediterranean

Background:Gastrointestinal involvement(GI) is a common feature of systemic sclerosis(SSc) and can be highly disabling, representing a major cause of morbidity and reduced quality of life(QoL). The impact of dietary habits on GI symptoms, mood and QoL has not been extensively evaluated.Objectives:To evaluate the adherence to the Mediterranean Diet(MD) in an Italian multicenter cohort of SSc patients, and its impact on GI symptoms and other disease features, depression, anxiety and overall QoL.Methods:Consecutive SSc(ACR/EULAR2013) patients from 4 Italian cohorts were enrolled. Dietary habits and adherence to the MD were assessed using the 14-item MEDAS and QueMD questionnaires. Presence and severity of depressive/anxious symptoms and QoL were evaluated with the Hospital Anxiety and Depression Scale(HADS) and the SSc-HAQ(S-HAQ). GI symptoms were assessed with the Reflux Disease Questionnaire(RDQ) and the UCLA SCTC GI Tract 2.0 questionnaire(USG). Associations with patients’ lifestyle, disease characteristics, and nutritional status were explored.Results:265 patients (94.7% females; age 55.8±13.6years; disease duration 9.1±7.0years; diffuse SSc 31.8%; Scl70 + 35.8%;ulcers 23.4%;ILD 29.4%;BMI 23.7±4.4 Kg/m2; obese 11.3%,overweight 23.4%,underweight 4.9%) were enrolled.Overall MD adherence was moderate(7.5±1.9) according to MEDAS and it correlated with QueMD score(4.53±1.96)(R=.371,p<0.001). MD adherence was optimal in 39 patients(14.7%), more frequently from Central-Southern Italy(p=0.036); 189 patients(71.3%) had a good and 37(14.0%) a poor MD adherence.GI symptoms were moderate/severe according to USG in 37(14.0%) patients(USG:0.41±0.40), and heartburn was the most common GI symptom(35.2%). The prevalence of significant anxiety and depression was 24.1% and 17.0%.An inverse correlation was found between MD adherence and mood disturbances at HADS(MEDAS; R=-0.181,p=0.04), work impairment(QueMD;R=-0.247,p=0.005) and reduced QoL, both for GI (constipation at USG: R=-0.133,p=0.032) and general S-HAQ items(bowel:R=-0.181,p=0.04;severity R=-0.202,p=0.01;Raynaud:R=-0.217,p<0.001;ulcers: R=-0.207,p=0.01). MD adherence directly correlated with lung function(MEDAS;R=0.181,p=0.023 for FVC and R=0.170,p=0.03 for DLCO). Patients with MD optimal adherence had lower HADS depression(p=0.04) and S-HAQ scores(HAQ,p=0.04; Raynaud,p=0.005; ulcers,p=0.02) and lower work impairment and lost work time(p=0.03). No significant correlation emerged between MD adherence and BMI, or specific symptoms. Depression and anxiety directly correlated with the severity of reported upper GI symptoms according to both scales (RDQ-GERD:R=0.261,p=0.001; USG:R=0.263,p<0.001) and general S-HAQ items (HAQ: R=0.136,p=0.032;severity R=0.233,p<0.001;bowel: R=0.135,p=0.04;breath: R=0.133,p=0.03; ulcers: R=0.132,p=0.037). Results were confirmed after exclusion of psychiatric(11.7%) and fibromyalgic(15.5%) patients.Conclusion:Unsatisfactory MD adherence is associated with a low mood, impaired QoL, work impairment, GI and vascular symptoms in Italian SSc patients. The promotion of a healthy lifestyle could positively impact on QoL and disease status of SSc patients.References:[1]Gnagnarella P, et a. NMCD 2018. DOI: 10.1016/j.numecd.2018.06.006[2]Jaeger VK et al. PLoS One, 2016.[3]Dinu M et al. Eur J Clin Nutr 2018. doi: 10.1038/ejcn.2017.58.[4]Khanna D, et al.Arthritis Rheum. 2009. doi: 10.1002/art.24730.Acknowledgments:GILSDisclosure of Interests:Giacomo De Luca Grant/research support from: SOBI, Speakers bureau: SOBI, Novartis, Pfizer, MSD, Celgene, Gerlando Natalello: None declared, Giuseppina Abignano: None declared, Corrado Campochiaro Speakers bureau: Novartis, Pfizer, Roche, GSK, SOBI, Duygu Temiz Karadağ: None declared, Maria De Santis: None declared, Elisa Gremese Speakers bureau: Abbvie, BMS, Celgene, Jannsen, Lilly, MSD, Novartis, Pfizer, Sandoz, UCB, Silvia Laura Bosello Speakers bureau: Abbvie, Pfizer, Boehringer, Lorenzo Dagna Grant/research support from: Abbvie, BMS, Celgene, Janssen, MSD, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, SG, SOBI, Consultant of: Abbvie, Amgen, Biogen, BMS, Celltrion, Novartis, Pfizer, Roche, SG, and SOBI

scholarly journals Quality of Life And Gerontology: Description of a Research Line

2014 ◽  
Vol 4 (3) ◽  
pp. 301-308
Author(s):  
Bernat-Carles Serdà Ferrer
Keyword(s):  
Quality Of Life ◽  
Therapeutic Option ◽  
Rehabilitation Program ◽  
Muscular Fitness ◽  
Psychological Mechanisms ◽  
Modular Concept ◽  
Specific Objective ◽  
Quasi Experimental ◽  
The Continuum

The Quality of Life (QoL) is a multidimensional, dynamic and modular concept. QoL is influenced by psychological mechanisms related to the adaptation disease. The aim of this paper is to describe the research line on QoL and chronic disease. The specific objective is to determine the mechanisms involved in the change of QoL in a group of elderly people with cancer. Method. The study is prospective quasi-experimental. With a sample of 130 participants with cancer. Results. The rehabilitation program improves muscular fitness, cardiovascular efficiency, (at rest and effort) and reduces the continued decline related to treatment. We have observed a significant increase in QoL in the period evaluated. This result confirms that QoL at baseline of the disease is low. Adherence to the program achieved is 93%. Conclusions. We confirm the effectiveness of a rehabilitation program tailored to the symptoms generated by the disease and cancer treatment in improving QoL of older people. The program is a therapeutic option to preserve the QoL of the patient in the continuum of the disease. Now we are studying the timing where the implementation of the program is most effective.

scholarly journals THU0632-HPR DETERMINANTS OF HAPPINESS AND QUALITY OF LIFE IN PEOPLE WITH SYSTEMIC SCLEROSIS: A STRUCTURAL EQUATION MODELLING APPROACH

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 561.1-561
Author(s):  
T. Santiago ◽  
E. Santos ◽  
A. C. Duarte ◽  
P. Martins ◽  
M. Sousa ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Systemic Sclerosis ◽  
Disease Activity ◽  
Personality Traits ◽  
Structural Equation Modelling ◽  
Structural Equation ◽  
Effective Control ◽  
Equation Modelling ◽  
Disease Impact

Background:In recent years more attention has been given to patients reported outcomes (PROs). Systemic sclerosis (SSc) is no exception. As there is no effective treatment or cure to SSc, it is important to recognize the relevance to patients of the different features of the disease to improve quality and enjoyment of life: the ultimate targets of therapy. Remarkably lacking in PROs is the evaluation of the overall perspective of subjective well being, equivalent to ‘happiness’ or “positive psychological dimensions”.Objectives:To examine the determinants of happiness and quality of life (QoL) in patients with SSc with emphasis on disease activity, disease impact and personality traits.Methods:This is an observational, cross-sectional and multicenter study from six rheumatology clinics in Portugal. A total of 113 patients with SSc with a complete set of data on disease activity, disease impact, personality, quality of life and happiness were included.Structural equation modelling (latent variable structural model) was used to estimate the association between the variables using a maximum likelihood estimation with Satorra-Bentler’s correction and performed with STATA® 15.0. Two hypotheses were pursued: H1 – Disease activity and impact of disease are negatively associated to overall QoL and happiness; H2 – ‘Positive’ personality traits are related to happiness both directly and indirectly through perceived disease impact.Results:Results obtained in the structural equation measurement model indicated a good fit [χ2/df=1.44; CFI=0.93; TLI=0.90; RMSEA=0.06] and supported all driving hypotheses (Figure 1). Happiness was positively related to ‘positive’ personality (β=0.45, p=0.01) and, to a lesser extent, negatively related with impact of disease (β=-0.32; p=0.01). This impact, in turn, was positively related to EUSTAR activity score (β=0.37; p<0.001) and mitigated by ‘positive’ personality traits (β=-0.57; p<0.001). Impact of disease had a much stronger relation with QoL than with happiness (β=-0.78, p<0.001). Quality of life and happiness had no statistically significant relationship.Conclusion:Optimization of Qol and happiness in people with SSc requires effective control of the disease process. Personality and its effects upon the patient´s perception of the disease impact, seems to play a pivotal mediating role in these relations and should deserve paramount attention if happiness and enjoyment of life is taken as the ultimate goal of health care.Disclosure of Interests:Tânia Santiago: None declared, Eduardo Santos: None declared, Ana Catarina Duarte: None declared, Patrícia Martins: None declared, Marlene Sousa: None declared, Franscisca Guimarães: None declared, Soraia Azevedo: None declared, Raquel Ferreira: None declared, Miguel Guerra: None declared, Ana Cordeiro Consultant of: Ana Cordeiro has acted as a consultant for Roche, Speakers bureau: Ana Cordeiro has received speaker fees from Boehringer Ingelheim, Lilly, and Vitoria, Inês Cordeiro: None declared, Sofia Pimenta: None declared, Patrícia Pinto: None declared, Maria Joao Salvador: None declared, José Antonio P. da Silva Grant/research support from: Pfizer, Abbvie, Consultant of: Pfizer, AbbVie, Roche, Lilly, Novartis

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