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Pulmonary hypertension and lupus

Vikas Pathak, Robert Aris, Brian C Jensen, Wei Huang, Hubert James Ford
BACKGROUND: Plasma pro-BNP (brain natriuretic peptide) levels are often elevated in response to right ventricular (RV) volume and pressure overload, parameters potentially affected by exercise. Plasma pro-BNP levels change in association with long-term changes in pulmonary hemodynamics, thereby serving as a potential biomarker in pulmonary arterial hypertension (PAH). The 6-min Walk Test (6MWT) and pro-BNP level are often checked in a single office visit. There is no universal standard for measuring Pro-BNP levels relative to the timing of the 6MWT...
March 21, 2018: Lung
Duo Huang, Yang-Yang Cheng, Pak-Hei Chan, Jojo Hai, Kai-Hang Yiu, Hung-Fat Tse, Ka-Lam Wong, Katherine Fan, Ying Wah Li, Woon-Leung Ng, Cheuk-Wan Yim, Cheuk-Hon John Wong, Lai-Shan Tam, Priscilla C H Wong, Chi-Yuen Wong, Chup-Hei Ho, Alexander M H Leung, Chi-Chiu Mok, Ho Lam, Chak-Sing Lau, Tommy Cheung, Carmen Ho, Sharon W Y Law, Esther W Chan, Li-Xue Yin, Wen-Sheng Yue, Toi Meng Mok, Mario Alberto Evora, Chung-Wah Siu
Current guideline-recommended screening for pulmonary hypertension in patients with systemic sclerosis has not been evaluated in systemic lupus erythematosus (SLE), which is disproportionately prevalent in Asians. This multicentre, cross-sectional screening study aims to study the prevalence of pulmonary hypertension among SLE patients using these guidelines, and identify independent predictors and develop a prediction model for pulmonary hypertension in SLE patients. SLE patients from participating centres will undergo an echocardiography- and biomarker-based pulmonary hypertension screening procedure as in the DETECT study...
January 2018: ERJ Open Research
Guillermo Pousada, Mauro Lago-Docampo, Adolfo Baloira, Diana Valverde
BACKGROUND AND OBJECTIVE: Pulmonary arterial hypertension associated with systemic lupus erythematosus (PAH-SLE) is a rare disease with a low incidence rate. In this study, PAH related genes and genetic modifiers were characterised molecularly in patients with PAH-SLE. PATIENTS AND METHODS: Three patients diagnosed with PAH-SLE and 100 control individuals were analysed after signing an informed consent. RESULTS: Two out of the three analysed patients with PAH-SLE were carriers of pathogenic mutations in the genes BMPR2 and ENG...
March 8, 2018: Medicina Clínica
Shu-Zhen Xu, Yan Liang, Xiang-Pei Li, Xiao-Mei Li, Zong-Wen Shuai, Rui-Xue Leng, Hai-Feng Pan, Dong-Qing Ye
Pulmonary arterial hypertension (PAH) is an increasingly recognized complication of systemic lupus erythematosus (SLE). This study aims to estimate the point prevalence of PAH and identify risk factors for PAH in a large cohort of hospitalized SLE patients. We have collected the medical records of patients hospitalized with SLE at the First Affiliated Hospital of Anhui Medical University and Anhui Provincial Hospital. Resting transthoracic echocardiography (TTE) was used to estimate pulmonary artery pressure (PAP) and PAH was defined as systolic PAP (PASP) > 30 mmHg...
March 8, 2018: Clinical Rheumatology
David Montani, Julien Henry, Caroline O'Connell, Xavier Jaïs, Vincent Cottin, David Launay, Gilbert Habib, Arnaud Bourdin, Mitja Jevnikar, Laurent Savale, Laurence Rottat, Gérald Simonneau, Olivier Sitbon, Marc Humbert, Yannick Allanore
BACKGROUND: Precapillary pulmonary hypertension (PH), and particularly pulmonary arterial hypertension (PAH), is a life-threatening complication of connective tissue diseases (systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease). The relationship between PH and rheumatoid arthritis (RA) has not been clearly established. OBJECTIVES: The aim of the study was to evaluate the relationship between precapillary PH and RA. METHODS: We identified patients with PH and suspected RA included in the French PH Registry between 1 May 2004 and 31 December 2012 and evaluated the prevalence of confirmed RA in this population...
February 28, 2018: Respiration; International Review of Thoracic Diseases
Lihua Sun, Ying Wang, Yu Dong, Shengda Song, Runlan Luo, Guangsen Li
OBJECTIVES: To assess right atrium (RA) function of patients with systemic lupus erythematosus (SLE) and pulmonary artery hypertension (PAH) by 2-dimensional speckle-tracking echocardiography. METHODS: Thirty matched healthy adults were selected as group A. Then, 102 patients with SLE were divided into 3 groups according to the severity of PAH. Group B included 37 patients without PAH (pulmonary artery [PA] systolic pressure ≤ 30 mm Hg); group C included 34 patients with PAH (PA systolic pressure of 30-50 mm Hg); and group D included 31 patients with PAH (PA systolic pressure ≥ 50 mm Hg)...
February 26, 2018: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
Isabel S Bazan, Kofi A Mensah, Anastasiia A Rudkovskaia, Percy K Adonteng-Boateng, Erica L Herzog, Lenore Buckley, Wassim H Fares
Pulmonary hypertension (PH) is a clinical syndrome that is subdivided into five groups per the World Health Organization (WHO) classification, based largely on hemodynamic and pathophysiologic criteria. WHO Group 1 PH, termed pulmonary arterial hypertension (PAH), is a clinically progressive disease that can eventually lead to right heart failure and death, and it is hemodynamically characterized by pre-capillary PH and increased pulmonary vascular resistance in the absence of elevated left ventricular filling pressures...
January 2018: Respiratory Medicine
Seema Kini, Chetan Vekhande, Vikram Londhey
Background: Cardiovascular manifestations are responsible for considerable morbidity and mortality in patients with SLE. A wide range of manifestations due to active lupus, like pericarditis, valvular affection, myocarditis, and less commonly pulmonary hypertension, are described. This study was undertaken to study cardiovascular manifestations in SLE, with a focus on echocardiography findings, in an urban Indian setting. Methodology: Fifty consecutive cases of SLE following up in the Rheumatology Clinic of TNMC and BYL Nair Charitable hospital, an Indian tertiary care hospital were studied...
November 2017: Journal of the Association of Physicians of India
Akio Kawabe, Kazuhisa Nakano, Yukiyo Aiko, Satoshi Aramaki, Takeshi Onoue, Dan Okura, Yoshiya Tanaka
Pregnancy in women with systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH) remains a high risk. We successfully managed a pregnancy in a patient with SLE-PAH. A 31-year-old pregnant woman with SLE-PAH had worsening PAH and SLE flare-up during pregnancy and a sudden increase in pulmonary arterial pressure after delivery. SLE-PAH was controlled by continuous intravenous epoprostenol and inhaled nitric oxide therapy combined with high-dose corticosteroids under close hemodynamic monitoring...
January 11, 2018: Internal Medicine
Jean Joel Bigna, Jean Jacques Noubiap, Jobert Richie Nansseu, Leopold Ndemnge Aminde
BACKGROUND: Despite the recent increasing worldwide attention towards pulmonary hypertension (PH), its epidemiology remains poorly described in Africa. Accordingly, we performed a systematic review and meta-analysis of PH prevalence, incidence and etiologies in Africa. METHODS: We searched PubMed, EMBASE, African Journals Online, and Africa Index Medicus. Published observational studies until September 20, 2017, including adult participants residing in Africa were considered...
December 8, 2017: BMC Pulmonary Medicine
Jeremy Fleri Soler, Andrew Borg, Cecilia Mercieca
A 32-year-old woman suffering from systemic lupus erythematosus presented with a 6-week history of progressive dyspnoea and pleuritic chest pain. Examination was normal apart from reduced air entry at the lung bases.Arterial blood gases showed hypoxaemia and chest X-ray revealed raised hemidiaphragms without any pleural effusions. Lung function showed a restrictive pathology while high-resolution chest CT and CT pulmonary angiogram were negative. Echocardiography showed normal ventricular diameters and no pericardial effusion...
November 8, 2017: BMJ Case Reports
Ayesha Zia, Joy Russell, Ravi Sarode, Surendranath R Veeram, Shellie Josephs, Kendra Malone, Song Zhang, Janna Journeycake
BACKGROUND: Sequelae of venous thromboembolism (VTE) in children include recurrence, development of post thrombotic syndrome (PTS) when venous return from a limb is affected and chronic thromboembolic pulmonary hypertension (CTEPH) after pulmonary embolism. Identification of laboratory-based risk factors may be useful for individualized risk assessment for VTE sequelae. Coagulation activation and inflammation may contribute to their pathophysiology. We performed a systematic review to investigate the association between biomarkers of coagulation activation, inflammation and fibrinolysis and adverse VTE outcomes in children and young adults...
December 2017: Thrombosis Research
G A Movsisyan, T V Martynyuk, K V Mershin, N M Danilov, M A Satybaldyeva, T M Reshetnyak, R S Akchurin, E L Nasonov, I Ye Chazova
Chronic thromboembolic pulmonary hypertension (CTEPH) is one of the potentially curable forms of pulmonary hypertension, in which pulmonary thromboendarterectomy is the gold standard treatment. However, over the last decade, great attention has been given to a combined therapeutic approach including both drug therapy and surgical treatment and the application of endovascular technologies. This clinical case demonstrates the diagnostic difficulties of CTEPH and the opportunities of a comprehensive approach to therapy for the disease with mandatory assessment of preoperative surgical and medical treatment in order to improve the patient status and to prepare for surgery...
2017: Terapevticheskiĭ Arkhiv
Hamza Jawad, Sebastian R McWilliams, Sanjeev Bhalla
PURPOSE OF REVIEW: The study aimed to illustrate the cardiopulmonary findings of the following collagen vascular diseases on cross-sectional imaging: rheumatoid arthritis, scleroderma (progressive systemic sclerosis), systemic lupus erythematosus, the inflammatory myopathies (polymyositis/dermatomyositis), and Sjögren's syndrome. RECENT FINDINGS: Although collagen vascular diseases can affect any part of the body, interstitial lung disease and pulmonary hypertension are the two most important cardiopulmonary complications and are responsible for the majority of morbidity and mortality in this patient population...
October 9, 2017: Current Rheumatology Reports
Leila Ghofraniha, Zahra Mirfeizi, Fatemeh Seyyedi Khabbaz, Farveh Vakilian, Saeed Eslami
INTRODUCTION: Pulmonary arterial hypertension (PAH) is an increasingly recognized complication of systemic lupus erythematous (SLE), which may remain undiagnosed if asymptomatic. OBJECTIVE: This study aimed to determine the correlation between echocardiographic findings of PAH and six-minute walk test (6WMT) and serum pro b-type natriuretic peptide (proBNP) level in patients with SLE. METHODS: This cross-sectional study was performed on 50 SLE patients selected from patients referring to the outpatient's department of the Rheumatology Clinic at Imam Reza Hospital in Mashhad, Iran, from July 2013 through September 2014, using resting transthoracic echocardiography to estimate systolic pulmonary artery pressure (sPAP)...
August 2017: Electronic Physician
Martin Fortin, Jeannie Haggerty, Steven Sanche, José Almirall
BACKGROUND: Various data sources may be used to document the presence of chronic medical conditions. This study examined the agreement between self-reported and health administrative data. METHODS: A randomly selected cohort of participants aged 25-75 years recruited by telephone from the general population of Quebec reported on the presence of 1 or more chronic conditions from a candidate list of 12 conditions: diabetes, hypertension, thyroid disorder, any cardiac disease, cancer diagnosis in the previous 5 years (including melanoma but excluding other skin cancers), asthma, osteoarthritis, rheumatoid arthritis or lupus, osteoporosis, chronic obstructive pulmonary disease, intestinal disease and hypercholesterolemia...
September 25, 2017: CMAJ Open
Rukma Parthvi, Rutuja R Sikachi, Abhinav Agrawal, Ajay Adial, Abhinav Vulisha, Sameer Khanijo, Arunabh Talwar
Antiphospholipid (aPL) antibodies are antibodies specific for anionic phospholipids. They are immunoglobulins that attack phospholipids, phospholipid-binding proteins, or phospholipid-protein complexes and are detected in anticardiolipin and lupus anticoagulant assays. aPL antibodies are often associated with antiphospholipid syndrome (APS) which can be idiopathic or from secondary causes such as systemic lupus erythematosus (SLE), infection or drugs. They have also been shown to be associated with Pulmonary Hypertension...
August 2017: Intractable & Rare Diseases Research
Hidekata Yasuoka, Yuichiro Shirai, Yuichi Tamura, Tsutomu Takeuchi, Masataka Kuwana
BACKGROUND: The potential efficacy of immunosuppressive (IS) treatment has been reported in patients with pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD), but its positioning in the treatment algorithm remains uncertain. The aim of this study was to identify predictors of favorable responses to first-line IS treatment.Methods and Results:This single-center retrospective study included 30 patients with PAH accompanied by systemic lupus erythematosus (SLE), mixed CTD (MCTD), or primary Sjögren's syndrome (SS) who received first-line IS treatment alone or in combination with pulmonary vasodilators...
September 12, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
Eric Hachulla, Xavier Jais, Gaël Cinquetti, Pierre Clerson, Laurence Rottat, David Launay, Vincent Cottin, Gilbert Habib, Grégoire Prevot, Céline Chabanne, Eléna Foïs, Zahir Amoura, Luc Mouthon, Véronique Le Guern, David Montani, Gérald Simonneau, Marc Humbert, Vincent Sobanski, Olivier Sitbon
BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare complication of systemic lupus erythematosus (SLE). METHODS: We identified all patients with SLE and PAH (SLE-PAH) who were enrolled in the French Pulmonary Hypertension Registry with a diagnosis confirmed by right heart catheterization (RHC). A control group of 101 patients with SLE without known PAH was selected from SLE expert centers participating in the Pulmonary Hypertension Registry. Survival was estimated by the Kaplan-Meier method...
August 26, 2017: Chest
Sean Gaine, Kelly Chin, Gerry Coghlan, Richard Channick, Lilla Di Scala, Nazzareno Galiè, Hossein-Ardeschir Ghofrani, Irene M Lang, Vallerie McLaughlin, Ralph Preiss, Lewis J Rubin, Gérald Simonneau, Olivier Sitbon, Victor F Tapson, Marius M Hoeper
Patients with connective tissue disease-associated pulmonary arterial hypertension (PAH-CTD) have a poor prognosis compared with other aetiologies. The underlying CTD can influence treatment response and outcomes. We characterised the GRIPHON study PAH-CTD subgroup and evaluated response to selexipag.Of 334 patients with PAH-CTD, PAH was associated with systemic sclerosis (PAH-SSc) in 170, systemic lupus erythematosus (PAH-SLE) in 82 and mixed CTD/CTD-other in 82. For the primary composite endpoint of morbidity/mortality, hazard ratios (HR) and 95% CI were calculated using Cox proportional hazard models...
August 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
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