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Pulmonary hypertension lupus

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https://www.readbyqxmd.com/read/28818881/selexipag-for-the-treatment-of-connective-tissue-disease-associated-pulmonary-arterial-hypertension
#1
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
https://www.readbyqxmd.com/read/28790444/anti-smd1-antibodies-are-associated-with-renal-disorder-seizures-and-pulmonary-arterial-hypertension-in-chinese-patients-with-active-sle
#2
Chaojun Hu, Mengtao Li, Jian Liu, Junyan Qian, Dong Xu, Shulan Zhang, Ping Li, Jiulang Zhao, Xinping Tian, Xiaofeng Zeng
Detection of autoantibodies in systemic lupus erythematosus (SLE) plays an important role in timely diagnosis and earlier treatment of SLE. In this study, we used a SmD1 polypeptide-based ELISA to determine anti-SmD1 antibody in 269 SLE, including100 naïve (had not been treated with steroids or immunosuppressants at study inception) SLE patients and 169 non-naive SLE patients; 233 controls with other rheumatic diseases (RDC) (70 RA, 40 AS, 73SSc, and 50 SS), and 110 healthy controls (HC) group. The positive rate of anti-SmD1 among all SLE patients was 60...
August 8, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28748509/clinical-characteristics-and-thrombosis-outcomes-of-paediatric-antiphospholipid-syndrome-analysis-of-58-patients
#3
Jingran Ma, Hongmei Song, Min Wei, Yanyan He
The study aims to analyse the clinical and immunological manifestations of paediatric antiphospholipid syndrome (APS) in patients, based on the 2006 revised classification criteria of definite APS. Fifty-eight paediatric patients with APS were enrolled and analysed retrospectively. A total of 37 female and 21 male patients with a mean age of 14 ± 3 years at disease onset were included. Fourteen (24%) cases were primary APS, and 40 (69%) cases were secondary to systemic lupus erythaematosus (SLE). Anti-nuclear antibody (ANA) positivity and hypocomplementemia were more common in secondary APS than in primary APS...
July 26, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28704174/evaluation-of-pulmonary-artery-pressure-in-patients-with-juvenile-systemic-lupus-erythematosus-sle
#4
Amra Adrovic, Reyhan Dedeoglu, Sezgin Sahin, Kenan Barut, Aida Koka, Dicle Cengiz, Funda Oztunc, Ozgur Kasapcopur
Juvenile systemic lupus erythematosus (jSLE) is a chronic multisystemic autoimmune disease. Previous studies among adults have shown impaired right ventricular (RV) function in patients with SLE. Also, these patients may develop pulmonary artery hypertension (PAH), which is one of the most threatening complications of SLE. Nevertheless, studies on PAH among jSLE patients are still rare. The aim of this study was to assess the RV function in jSLE patients by Doppler echocardiography (Echo Doppler). We also estimated pulmonary artery systolic pressure (PASP) and mean pulmonary artery pressure (MPAP) in these patients...
July 13, 2017: Bosnian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/28675998/cardiovascular-and-pulmonary-manifestations-of-systemic-lupus-erythematosus
#5
M B Urowitz, Konstantinos Tselios
BACKGROUND: Systemic lupus erythematosus (SLE) is characterized by various clinical manifestations and immunologic abnormalities. Among clinical manifestations, cardiovascular and respiratory system involvement is increasingly recognized as critical for patients' prognosis. OBJECTIVE: Heart involvement, most commonly presents with pericarditis. However, valvular disease and less often myocarditis may be detected. METHOD: Accelerated atherosclerosis is currently considered as one of the most important co-morbidities of SLE with cardiovascular events being one of the leading causes of death at relatively young ages...
July 4, 2017: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/28650277/attenuated-post-exercise-heart-rate-recovery-in-patients-with-systemic-lupus-erythematosus-the-role-of-disease-severity-and-beta-blocker-treatment
#6
P Bienias, M Ciurzyński, A Chrzanowska, I Dudzik-Niewiadomska, K Irzyk, K Oleszek, A Kalińska-Bienias, B Kisiel, W Tłustochowicz, P Pruszczyk
Objective Sinus tachycardia is frequently reported in systemic lupus erythematosus (SLE), while there are limited data on post-exercise ability to slow heart rate (i.e. heart rate recovery, HRR) in this group of patients. Methods We studied consecutive 70 patients with SLE and 30 healthy controls. All examined individuals underwent detailed clinical examination, echocardiography, Holter monitoring with heart rate variability and treadmill stress test using Bruce's protocol. HRR values were calculated as the difference between maximum HR during exercise and HR at the first (HRR1) and third (HRR3) minute of rest...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28639945/pulmonary-arterial-hemodynamic-assessment-by-a-novel-index-in-systemic-lupus-erythematosus-patients-pulmonary-pulse-transit-time
#7
Tolga Han Efe, Mehmet Doğan, Cem Özişler, Tolga Çimen, Mehmet Ali Felekoğlu, Ahmet Göktuğ Ertem, Engin Algül, Sadık Açıkel
OBJECTIVE: Systemic lupus erythematosus (SLE) is a chronic, inflammatory, and autoimmune connective tissue disease. One of the leading causes of mortality among SLE patients is pulmonary hypertension. The aim of this study was to evaluate the association between echocardiographic findings, including the pulmonary pulse transit time and pulmonary hypertension parameters, in SLE patients. METHODS: Thirty SLE patients (aged 39.9±11 years, 28 females) as the study group and 34 age- and sex-matched healthy volunteers (aged 37...
June 22, 2017: Anatolian Journal of Cardiology
https://www.readbyqxmd.com/read/28606232/-a-clinical-analysis-of-15-children-with-systemic-lupus-erythematosus-accompanied-by-pulmonary-hypertension
#8
Ji Li, Jing-Ran Ma, Zhi-Xing Sun, Jing-Jing Jiang, Yan-Qing Dong, Qian Wang, Hong-Mei Song
OBJECTIVE: To evaluate the clinical features, laboratory findings, diagnosis and treatment, and prognosis of children with systemic lupus erythematosus (SLE) accompanied by pulmonary hypertension (PH). METHODS: The clinical symptoms, laboratory findings, echocardiographic features, SLE disease activity index, and treatment outcome of 15 hospitalized children with SLE accompanied by PH were retrospectively analyzed. RESULTS: Among the 15 patients, the median interval from diagnosis of SLE to diagnosis of PH was 0...
June 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28546762/cardiovascular-magnetic-resonance-imaging-clinical-implications-in-the-evaluation-of-connective-tissue-diseases
#9
REVIEW
Sophie Mavrogeni, George Markousis-Mavrogenis, Loukia Koutsogeorgopoulou, Genovefa Kolovou
Cardiovascular magnetic resonance imaging is a recently developed noninvasive, nonradiating, operator-independent technique that has been successfully used for the evaluation of congenital heart disease, valvular and pericardial diseases, iron overload, cardiomyopathies, great and coronary vessel diseases, cardiac inflammation, stress-rest myocardial perfusion, and fibrosis. Rheumatoid arthritis and other spondyloarthropathies, systemic lupus erythematosus, inflammatory myopathies, mixed connective tissue diseases (CTDs), systemic sclerosis, vasculitis, and sarcoidosis are among CTDs with serious cardiovascular involvement; this is due to multiple causative factors such as myopericarditis, micro/macrovascular disease, coronary artery disease, myocardial fibrosis, pulmonary hypertension, and finally heart failure...
2017: Journal of Inflammation Research
https://www.readbyqxmd.com/read/28538274/prevalence-of-pulmonary-embolism-among-systemic-lupus-erythematosus-discharges-a-decade-of-analysis-of-the-national-hospital-discharge-survey
#10
Srinadh Annangi, Tirumala Rao Dammalapati, Snigdha Nutalapati, Marshaleen N Henriques King
INTRODUCTION: Pulmonary embolism (PE) is a life threatening preventable medical condition involving sudden occlusion of arteries within the lungs. Systemic lupus erythematosus (SLE) is an inflammatory disorder and therefore independently poses a risk of PE. We aimed to determine the association of SLE and PE using National Hospital Discharge Survey data, a national representative sample of hospital discharges throughout the United States. METHODS: Retrospective population-based analysis was done using National Hospital Discharge Survey data for the period 2001 to 2010...
June 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/28515936/pulse-dose-steroids-in-severe-pulmonary-arterial-hypertension-secondary-to-systemic-lupus-erythematosus
#11
Cody Lee, Jean Elwing
OBJECTIVE: The pulmonary vascular targeted treatment for systemic lupus erythematosus-associated pulmonary arterial hypertension is similar to other connective tissue disease-associated pulmonary arterial hypertension. In addition, there also appears to be a role for immunosuppression in the overall management. However, the optimal immunosuppressive regimen and what patients will respond to treatments are currently not clearly elucidated given the lack of randomized controlled trials on the subject...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28420842/the-development-of-marked-collateral-circulation-due-to-inferior-vena-cava-filter-occlusion-in-a-patient-with-chronic-thromboembolic-pulmonary-hypertension-complicated-with-anti-phospholipid-syndrome
#12
Hajime Kasai, Nobuhiro Tanabe, Ken Koshikawa, Yasutaka Hirasawa, Toshihiko Sugiura, Seiichiro Sakao, Koichiro Tatsumi
A 30-year-old Japanese man was diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH) with lupus anticoagulants (LAs) in 2003. He underwent pulmonary endarterectomy after the placement of an inferior vena cava filter (IVCF) in 2004, and treatment with warfarin was continued. In 2014, IVCF occlusion and marked collateral circulation were noted during an examination for transient dyspnea; however, his warfarin level was within the therapeutic range for 88.9% of the time from 2003 to 2014. We herein report a rare case of CTEPH and LAs with IVCF occlusion; in such cases, intense treatment may be required...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28411166/antiphospholipid-antibodies-can-identify-lupus-patients-at-risk-of-pulmonary-hypertension-a-systematic-review-and-meta-analysis
#13
REVIEW
Stéphane Zuily, Vinicius Domingues, Christine Suty-Selton, Valérie Eschwège, Laurent Bertoletti, Ari Chaouat, François Chabot, Véronique Regnault, Evelyn M Horn, Doruk Erkan, Denis Wahl
BACKGROUND: Pulmonary hypertension (PH) is a life-threatening condition that may affect outcomes in patients with systemic lupus erythematosus (SLE). The role of antiphospholipid antibodies (aPL) on the risk of PH is controversial. Therefore our objective was to estimate the risk of PH (WHO groups 1-5) including associated pulmonary arterial hypertension (APAH, WHO group 1 only) related to aPL in patients with SLE. METHODS: Systematic review and meta-analysis were performed: MEDLINE, EMBASE, Cochrane Library, congress abstracts, and reference lists of eligible studies were searched through 2015...
April 12, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28409522/serological-biomarkers-as-risk-factors-of-sle-associated-pulmonary-arterial-hypertension-a-systematic-review-and-meta-analysis
#14
J Wang, J Qian, Y Wang, J Zhao, Q Wang, Z Tian, M Li, X Zeng
Objective This article aims to determine the serological biomarkers which can be considered as risk factors of systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension by a systematic review and meta-analysis. Methods This study was conducted in accordance with the PRISMA statement. The search database included MEDLINE, EMBASE, Cochrane Library and Scopus. The Newcastle-Ottawa scale was used for the quality assessment. The odds ratio was the primary measure of effect of the risk factors...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28293128/exhaled-nitric-oxide-in-systemic-sclerosis-lung-disease
#15
Natalie K Kozij, John T Granton, Philip E Silkoff, John Thenganatt, Shobha Chakravorty, Sindhu R Johnson
Background. Exhaled nitric oxide (eNO) is a potential biomarker to distinguish systemic sclerosis (SSc) associated pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD). We evaluated the discriminative validity, feasibility, methods of eNO measurement, and magnitude of differences across lung diseases, disease-subsets (SSc, systemic lupus erythematosus), and healthy-controls. Methods. Consecutive subjects in the UHN Pulmonary Hypertension Programme were recruited. Exhaled nitric oxide was measured at 50 mL/s intervals using chemiluminescent detection...
2017: Canadian Respiratory Journal: Journal of the Canadian Thoracic Society
https://www.readbyqxmd.com/read/28216071/antiphospholipid-antibodies-disappearance-in-primary-antiphospholipid-syndrome-thrombosis-recurrence
#16
REVIEW
Gabriela Medina, Eduardo Briones-García, María Pilar Cruz-Domínguez, Oscar I Flórez-Durante, Luis J Jara
OBJECTIVE: To evaluate the clinical outcome after aPL (antiphospholipid antibodies) disappearance in primary APS patients. METHODS: From a cohort of 70 patients with primary APS, we selected patients with positive aPL determinations at onset and ≥2 subsequent negative aPL determinations during the last 5years. To corroborate the immunologic profile, we determined IgG/IgM aCL antibodies, IgG/IgM antiβ2GPl, anti-annexin A5 antibodies and lupus anticoagulant (LA)...
April 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28205373/pulmonary-hypertension-in-connective-tissue-diseases-an-update
#17
REVIEW
Ramya Aithala, Anoop G Alex, Debashish Danda
Pulmonary hypertension (PH) is a relatively commoner complication of systemic sclerosis (SSc) with estimated prevalence ranging between 8% and 12% as compared to much lower figures in other connective tissue diseases (CTD). It is a major cause of morbidity and mortality in CTDs. PH is classified into five major groups. CTD-associated PH belongs to group 1 PH, also known as pulmonary arterial hypertension (PAH). Around 30% of scleroderma-related deaths are due to PAH. Underlying pathogenesis is related to pulmonary vasculopathy involving small vessels...
January 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28202869/treatment-of-vasodilator-resistant-mixed-connective-tissue-disease-associated-pulmonary-arterial-hypertension-with-glucocorticoid-and-cyclophosphamide
#18
Eri Sugawara, Masaru Kato, Ryo Hisada, Kenji Oku, Toshiyuki Bohgaki, Tetsuya Horita, Shinsuke Yasuda, Tatsuya Atsumi
Pulmonary arterial hypertension (PAH) associated with systemic lupus erythematosus (SLE) or mixed connective tissue disease (MTCD), in contrast to other types of PAH, may respond to immunosuppressive therapy. Most PAH cases with an immunosuppressant response were in the early stages of the disease (WHO functional class III or less). The present case was a 34-year-old woman with MCTD-associated PAH (WHO functional class IV) who was resistant to a combination of three vasodilators. Afterwards, she was treated with glucocorticoid and cyclophosphamide...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28164513/baseline-red-blood-cell-distribution-width-correlates-with-disease-activity-and-therapeutic-outcomes-in-patients-with-systemic-lupus-erythematosus-irrespective-of-anemia-status
#19
Xing-Li Zou, Xiao-Jing Lin, Xun Ni, Jing Wang, Wen Liu, Jin Wei
BACKGROUND: Red blood cell distribution width (RDW) has been recently found to reflect systemic inflammation in addition to anisocytosis, and its value for assessing disease activity of systemic lupus erythematosus (SLE) has been addressed in two studies, but its correlation with therapeutic outcomes and disease flare has not been evaluated. METHODS: One hundred and ninety-six newly diagnosed patients with SLE (all-SLE), including 105 non-anemic patients (na-SLE) and 91 patients with anemia (a-SLE) were prospectively studied...
October 1, 2016: Clinical Laboratory
https://www.readbyqxmd.com/read/28159359/clinical-characteristics-and-survival-of-pulmonary-arterial-hypertension-associated-with-three-major-connective-tissue-diseases-a-cohort-study-in-china
#20
Jiuliang Zhao, Qian Wang, Yongtai Liu, Zhuang Tian, Xiaoxiao Guo, Hui Wang, Jinzhi Lai, Can Huang, Xiaoxi Yang, Mengtao Li, Xiaofeng Zeng
OBJECTIVE: Pulmonary arterial hypertension (PAH) is a major cause of death in connective tissue disease patients. This study investigated the clinical characteristics and survival of CTD-PAH in Chinese patients. METHODS: This cohort study enrolled 190 consecutive PAH patients with systemic lupus erythematosus (SLE), systemic sclerosis (SSc), or primary Sjögren's syndrome (pSS) who visited our referral center between May 2006 and December 2014. Baseline demographics, clinical features, laboratory results, and hemodynamic assessments were analyzed...
June 1, 2017: International Journal of Cardiology
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