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Pulmonary hypertension lupus

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https://www.readbyqxmd.com/read/28216071/antiphospholipid-antibodies-disappearance-in-primary-antiphospholipid-syndrome-thrombosis-recurrence
#1
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)...
February 13, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28205373/pulmonary-hypertension-in-connective-tissue-diseases-an-update
#2
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...
February 16, 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
#3
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
#4
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
#5
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...
January 18, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28155616/crosstalk-between-oxidative-and-nitrosative-stress-and-arterial-stiffness
#6
Ioana Mozos, Constantin Tudor Luca
Arterial stiffness, the expression of reduced arterial elasticity, is an effective predictor of cardiovascular disorders. Oxidative stress is an imbalance between exposure to toxic reactive oxygen species (ROS) and antioxidant systems. The increase in reactive nitrogen species (RNS) is termed nitrosative stress. We review the main mechanisms and products linking arterial stiffness with oxidative and nitrosative stress in several disorders, focusing on recent experimental and clinical data, and the mechanisms explaining benefits of antioxidant therapy...
February 1, 2017: Current Vascular Pharmacology
https://www.readbyqxmd.com/read/28072955/-clinical-analysis-of-12-patients-with-pediatric-antiphospholipid-syndrome-with-pulmonary-embolism
#7
J R Ma, H M Song, J Xiao, X Y Tang, Y Y He, M Wei
Objective: To identify the clinical and immunological characteristics of pediatric antiphospholipid syndrome (APS) patients with pulmonary embolism. Method: Among 47 pediatric APS patients from Peking Union Medical College Hospital during the year of 2000 to 2015, 12 patients were diagnosed of pulmonary embolism, who were investigated and compared with APS patients without pulmonary embolism. Result: Twelve patients (among whom 6 cases were primary and the other 6 were secondary APS)had pulmonary embolism and all of them were non-shock type, which was the first presenting manifestation in 6 of them...
January 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28053559/systemic-lupus-erythematosus-and-pulmonary-arterial-hypertension-links-risks-and-management-strategies
#8
REVIEW
Konstantinos Tselios, Dafna D Gladman, Murray B Urowitz
Systemic lupus erythematosus (SLE) is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH), after systemic sclerosis, among the connective tissue diseases. SLE-associated PAH is hemodynamically defined by increased mean pulmonary artery pressure at rest (≥25 mmHg) with normal pulmonary capillary wedge pressure (≤15 mmHg) and increased pulmonary vascular resistance. Estimated prevalence ranges from 0.5% to 17.5% depending on the diagnostic method used and the threshold of right ventricular systolic pressure in studies using transthoracic echocardiogram...
2017: Open Access Rheumatology: Research and Reviews
https://www.readbyqxmd.com/read/27826712/subclinical-pulmonary-hypertension-in-childhood-systemic-lupus-erythematosus-associated-with-minor-disease-manifestations
#9
Pedro Anuardo, Monica Verdier, Natali W S Gormezano, Gabriela R V Ferreira, Gabriela N Leal, Alessandro Lianza, Juliana C O A Ferreira, Rosa M R Pereira, Nadia E Aikawa, Maria Teresa Terreri, Claudia S Magalhães, Simone Appenzeller, Maria Carolina Dos Santos, Silvana B Sachetti, Claudio A Len, Gecilmara S Pilleggi, Simone Lotufo, Eloisa Bonfá, Clovis A Silva
The aim of this study was to evaluate pulmonary hypertension (PH) in 852 childhood-onset systemic lupus erythematosus (cSLE) patients. This was a large multicenter study conducted in 10 Pediatric Rheumatology Services of São Paulo state, Brazil. PH was defined as systolic pulmonary artery pressure >35 mmHg and/or measurement of the mean pulmonary artery pressure >25 mmHg and/or diastolic pressure >15 mmHg by transthoracic echocardiogram. Demographic data, clinical manifestations, disease activity score (SLEDAI-2K), disease damage score (SLICC/ACR-DI) and treatments were also evaluated...
November 8, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27799439/systemic-lupus-erythematosus-in-spanish-males-a-study-of-the-spanish-rheumatology-society-lupus-registry-relesser-cohort
#10
A Riveros Frutos, I Casas, I Rúa-Figueroa, F J López-Longo, J Calvo-Alén, M Galindo, A Fernández-Nebro, J M Pego-Reigosa, A Olivé Marqués
OBJECTIVE: The objective of this study was to describe the demographic, clinical, and immunological manifestations of systemic lupus erythematosus (SLE) in male patients. METHODS: A cross-sectional, multicenter study was carried out of 3651 patients (353 men, 9.7%, and 3298 women, 90.2%) diagnosed with SLE, included in the Spanish Rheumatology Society SLE Registry (RELESSER). RESULTS: Mean ages (18-92 years) of symptom onset were 37 (SD 17) years (men) and 32 (SD 14) years (women)...
October 31, 2016: Lupus
https://www.readbyqxmd.com/read/27708976/the-clinical-significance-of-antiphospholipid-antibodies-in-systemic-lupus-erythematosus
#11
REVIEW
Ozan Ünlü, Stephane Zuily, Doruk Erkan
Antiphospholipid syndrome (APS) is the association of thrombosis and/or pregnancy morbidity with antiphospholipid antibodies (aPL). Thirty to forty percent of systemic lupus erythematosus (SLE) patients are tested positive for aPL, which may have an impact on the SLE presentation, management, and prognosis. Compared with SLE patients without aPL, those with aPL have a higher prevalence of thrombosis, pregnancy morbidity, valve disease, pulmonary hypertension, livedo reticularis, thrombocytopenia, hemolytic anemia, acute/chronic renal vascular lesions, and moderate/severe cognitive impairment; worse quality of life; and higher risk of organ damage...
June 2016: European Journal of Rheumatology
https://www.readbyqxmd.com/read/27702930/triple-threat-in-pregnancy
#12
Swati Vishwanathan, Michael Lucke, Indu G Poornima
A woman aged 22 years with a history of lupus presented in the 18th week of pregnancy with hypertensive emergency and flash pulmonary oedema. Bedside echocardiogram revealed severe left ventricular (LV) dysfunction with an ejection fraction (EF) of 25% and pericardial effusion. Laboratories revealed hypocomplementemia, proteinuria, elevated C reactive protein and anti-DS-DNA, raising concern for a lupus flare. Cardiac MRI showed an acute intramyocardial oedematous process, consistent with lupus carditis, and further worsening of LVEF to 13%...
October 4, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27687024/complementary-role-of-cardiovascular-imaging-and-laboratory-indices-in-early-detection-of-cardiovascular-disease-in-systemic-lupus-erythematosus
#13
S Mavrogeni, L Koutsogeorgopoulou, T Dimitroulas, G Markousis-Mavrogenis, G Kolovou
BACKGROUND: Cardiovascular disease (CVD) has been documented in >50% of systemic lupus erythematosus (SLE) patients, due to a complex interplay between traditional risk factors and SLE-related factors. Various processes, such as coronary artery disease, myocarditis, dilated cardiomyopathy, vasculitis, valvular heart disease, pulmonary hypertension and heart failure, account for CVD complications in SLE. METHODS: Electrocardiogram (ECG), echocardiography (echo), nuclear techniques, cardiac computed tomography (CT), cardiovascular magnetic resonance (CMR) and cardiac catheterization (CCa) can detect CVD in SLE at an early stage...
September 29, 2016: Lupus
https://www.readbyqxmd.com/read/27617783/-2016%C3%A2-review-on-catastrophic-antiphospholipid-syndrome
#14
REVIEW
Nathalie Costedoat-Chalumeau, Laetitia Coutte, Véronique Le Guern, Nathalie Morel, Gaelle Leroux, Romain Paule, Luc Mouthon, Jean-Charles Piette
The catastrophic antiphospholipid syndrome (CAPS) develops in at least 1% of patients with antiphospholipid syndrome, either primary or associated with systemic lupus erythematosus. CAPS reveals the antiphospholipid syndrome in about 50% of cases. The CAPS is characterized by rapidly-progressive widespread thromboses mainly affecting the microvasculature in the presence of antiphospholipid antibodies. In a few days, the patients develop multiorgan failure with renal insufficiency with severe hypertension, pulmonary, cerebral, cardiac, digestive and/or cutaneous involvement...
December 2016: La Presse Médicale
https://www.readbyqxmd.com/read/27571001/chronic-thromboembolic-pulmonary-hypertension-epidemiology-and-risk-factors
#15
Marion Delcroix, Kim Kerr, Peter Fedullo
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of pulmonary embolism. As for most rare diseases, epidemiologic data are scarce, but recent registries suggest an incidence of at least 5 per million inhabitants per year. A history of massive or recurrent acute pulmonary embolism is observed in most patients with CTEPH, but the proportion of patients who develop CTEPH after acute pulmonary embolism is a matter of debate, further complicated by the possible misdiagnosis of CTEPH as acute pulmonary embolism...
July 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27421214/connective-tissue-disease-related-pulmonary-arterial-hypertension
#16
REVIEW
Vivek Thakkar, Edmund M T Lau
Over the past two decades, there have been several advances in the assessment and management of connective tissue disease-related pulmonary arterial hypertension (CTD-PAH) that improved outcomes of the treatment of this lethal disease, and this will be the focus of this study. Systemic sclerosis is the leading cause of CTD-PAH, followed by systemic lupus erythematosus, mixed connective tissue disease, idiopathic inflammatory myositis, rheumatoid arthritis, and Sjogren's syndrome. Clinical registries have been invaluable in informing about the burden of disease, risk and prognostic factors, and temporal trends with respect to treatment and outcome in CTD-PAH...
February 2016: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/27390939/left-ventricular-diastolic-dysfunction-and-increased-left-ventricular-mass-index-related-to-pulmonary-hypertension-in-patients-with-systemic-autoimmune-disease-without-pericardial-effusion
#17
Atsushi Sugiura, Nobusada Funabashi, Koya Ozawa, Yoshio Kobayashi
PURPOSE: We investigated the relationship of left ventricular (LV) diastolic dysfunction and LV mass index (LVMI) against pulmonary hypertension (PH) in systemic autoimmune disease (SAD). METHODS: A total of 84 SAD patients (68 females; 53±17years; systemic lupus erythematosus, 27%; scleroderma, 17%; vasculitis, 16%; mixed connective tissue disease, 13% and polymyositis/dermatomyositis complex, 10%) without significant pericardial effusion (PE) on TTE (Vivid E9, GE) were analyzed...
October 1, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27143190/-the-clinical-manifestations-and-thrombotic-risk-factors-in-primary-antiphospholipid-syndrome
#18
J L Zhao, Y D Sun, Y Zhang, D Xu, Q Wang, M T Li, X F Zeng
OBJECTIVE: To investigate the clinical characteristics in patients with primary antiphospholipid syndrome (PAPS) and to identify potential predictors of thrombotic events. METHODS: A total of 107 patients with PAPS were enrolled in our study, who were admitted in Peking Union Medical College Hospital from January 2004 to December 2014. Demographic data, age at onset, disease duration, past history of hypertension and regular cigarette smoking, clinical manifestations, imaging characteristics, management and prognosis were retrospectively collected...
May 1, 2016: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/27006850/ana-negative-systemic-lupus-erythematosus-leading-to-cteph-ttp-like-thrombocytopenia-and-skin-ulcers
#19
Khalid Hamid Changal, Fayaz Sofi, Sheikh Shoaib Altaf, Adnan Raina, Ab Hameed Raina
SLE affects almost every organ system, with differing degrees of severity. During its clinical course periods of flares may alternate with periods of remission culminating in disease and therapy related damage. We describe a case of ANA negative SLE with severe thrombocytopenia, cutaneous vasculitis, antiphospholipid antibody syndrome, and pulmonary artery hypertension. As there is no definitive cure for SLE the treatment lies in caring for the individual organ systems involved and simultaneously taking care of the patient as a whole...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/26962774/baseline-characteristics-and-risk-factors-of-pulmonary-arterial-hypertension-in-systemic-lupus-erythematosus-patients
#20
Can Huang, Mengtao Li, Yongtai Liu, Qian Wang, Xiaoxiao Guo, Jiuliang Zhao, Jinzhi Lai, Zhuang Tian, Yan Zhao, Xiaofeng Zeng
Peking Union Medical College Hospital (PUMCH) has started a single-center right heart catheterization (RHC)-based pulmonary arterial hypertension (PAH) study in systemic lupus erythematosus (SLE) since 2006. The baseline characteristics of these patients were described and the risk factor for PAH in lupus was identified.The demographic, clinical, laboratory, and treatment characteristics of SLE patients with PAH when they were registered were collected as the baseline data. A case-control study was conducted by taking the admitted SLE-non-PAH patients adjusted for age and gender in a 4:1 ratio during the same period as the controls...
March 2016: Medicine (Baltimore)
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