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Pulmonary hypertension and lupus

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: Eur J Rheumatol
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
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
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...
September 9, 2016: La Presse Médicale
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
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
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
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]
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
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)
Hui Wang, Xiaoxiao Guo, Jinzhi Lai, Qian Wang, Zhuang Tian, Yongtai Liu, Mengtao Li, Jiuliang Zhao, Xiaofeng Zeng, Quan Fang
OBJECTIVES: Understanding health-related quality of life (HRQoL) is important in the management of patients with systemic lupus erythematosus associated pulmonary arterial hypertension (SLE-APAH), however, little is known about HRQoL and its determinants in these patients. METHODS: A total of 60 female SLE-APAH patients with mean age of 33.5 years were prospectively recruited from May 2013 to November 2014. Right heart catheter, SF-36 generic questionnaire, disease activity and functional status were assessed in all patients...
March 2016: Clinical and Experimental Rheumatology
Stephen C Mathai, Sonye K Danoff
The lung is a common site of complications of systemic connective tissue disease (CTD), and lung involvement can present in several ways. Interstitial lung disease (ILD) and pulmonary hypertension are the most common lung manifestations in CTD. Although it is generally thought that interstitial lung disease develops later on in CTD it is often the initial presentation ("lung dominant" CTD). ILD can be present in most types of CTD, including rheumatoid arthritis, scleroderma, systemic lupus erythematosus, polymyositis or dermatomyositis, Sjögren's syndrome, and mixed connective tissue disease...
2016: BMJ: British Medical Journal
A Najm, B Le Goff, A Achille, O Espitia, C Durant, F Perrin, C Agard
INTRODUCTION: Jaccoud's arthropathy (JA) is a chronic and non-erosive deforming arthropathy, usually affecting the hands. JA pathophysiology is poorly known but involves periarticular structures such as tendons and the joint capsule. JA is associated with various conditions including the connective tissue disease, especially systemic lupus erythematosis. JA has been rarely described and studied in systemic sclerosis. CASE REPORTS: We report the clinical histories of 3 patients with systemic sclerosis (ScS) who developed JA...
October 2016: La Revue de Médecine Interne
Jiri Vymetal, Martina Skacelova, Andrea Smrzova, Anna Klicova, Marketa Schubertova, Pavel Horak, Josef Zadrazil
BACKGROUND AND AIM: Rheumatic diseases are commonly considered chronic conditions. However, acute manifestations can be very severe and represent a diagnostic problem. Examples are systemic lupus erythematosus with acute flare, glomerulonephritis, CNS disorders and catastrophic antiphospholipid syndrome, scleroderma with interstitial lung disease, pulmonary hypertension and renal crisis and polyangiitis with alveolar haemorhage and acute respiratory failure. This aim of this paper is to overview emergency situations which can be encountered in the care of patients with autoimmune systemic diseases and vasculitides...
March 2016: Biomedical Papers of the Medical Faculty of the University Palacký, Olomouc, Czechoslovakia
L Fidler, K J Keen, Z Touma, S Mittoo
OBJECTIVES: This study examines the effect of pulmonary disease on patient-reported outcomes (PROs) and patient-performed outcome (PPO) in systemic lupus erythematosus (SLE) patients at a single tertiary referral center. METHODS: Pulmonary function tests (PFTs), chest imaging, SLE-related damage, and disease activity were examined in 110 SLE patients. Presence was noted of abnormal PFTs, pleural disease, pulmonary hypertension (PH), pulmonary infarction, interstitial lung disease (ILD), and shrinking lung syndrome (SLS)...
August 2016: Lupus
Joon Hyouk Choi, Seung-Jae Joo, Jinseok Kim
AIM: The prognosis of pulmonary hypertension (PH) in systemic sclerosis (SSC) and in systemic lupus erythematosus (SLE) is different. According to the guidelines, right heart catheterization (RHC) is necessary in pulmonary arterial hypertension (PAH) associated with connective tissue diseases (CTD). However, there is little supporting evidence. Therefore, we attempted to determine the necessity for RHC and the causes of differences in prognosis of PH by comparing SSC to SLE. METHODS: The inclusion criteria were all patients with SSC or SLE with exertional dyspnea...
January 2016: International Journal of Rheumatic Diseases
Chun-Ting Chen, Yu-Chen Tseng, Chih-Wei Yang, Hsuan-Hwai Lin, Peng-Jen Chen, Tien-Yu Huang, Yu-Lueng Shih, Wei-Kuo Chang, Tsai-Yuan Hsieh, Heng-Cheng Chu
The incidence of Sjögren syndrome (SS) in primary biliary cirrhosis (PBC) patients is high. The influence of SS on the clinical outcomes of PBC patients, however, remains unclear. Our study retrospectively collected data on PBC-only patients and PBC patients with concomitant SS (PBC-SS) to compare the clinical differences of long-term outcomes between them.A total of 183 patients were diagnosed with PBC from January 1999 to December 2014 at our hospital. Of these, the authors excluded patients with diabetes, hypertension, advanced liver cirrhosis at initial diagnosis of PBC (Child-Turcotte-Pugh classification score of ≥7) and other liver diseases (ie, alcoholic liver disease, alpha-antitrypsin deficiency, viral hepatitis, and primary sclerosing cholangitis), and autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis...
January 2016: Medicine (Baltimore)
J Zhao, W Bai, P Zhu, X Zhang, S Liu, L Wu, L Ma, L Bi, X Zuo, L Sun, C Huang, X Tian, M Li, Y Zhao, X Zeng
OBJECTIVES: To investigate both the prevalence and clinical characteristics of serositis in Chinese patients with systemic lupus erythematosus (SLE) in a large cohort in the Chinese SLE Treatment and Research group (CSTAR) database. METHODS: A prospective cross-sectional study of patients with SLE was conducted based on the data from the CSTAR registry. Serositis was defined according to the 1999 revised American College of Rheumatology (ACR) criteria for SLE - that is, pleuritis/pleural effusion and/or pericarditis/pericardial effusion detected by echocardiography, chest X-ray or chest computerized tomography (CT) scan...
May 2016: Lupus
Junyan Qian, Yanhong Wang, Can Huang, Xiaoxi Yang, Jiuliang Zhao, Qian Wang, Zhuang Tian, Mengtao Li, Xiaofeng Zeng
OBJECTIVE: This study aims to evaluate the survival of patients with systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH) by a systematic review and meta-analysis. METHODS: Studies were searched from MEDLINE (OVID), EMBASE, Cochrane Central Register of Controlled Trial and Scopus databases, and were selected according to the inclusion and exclusion criteria. Two independent reviewers extracted data from selected studies. Quality assessments were also performed using the Newcastle-Ottawa Scale...
March 2016: Autoimmunity Reviews
Karen M Olsson, Massimiliano Palazzini
The diverse challenges associated with diagnosis and management of patients with pulmonary hypertension are illustrated in this case-based review. Case 1 describes a patient diagnosed with pulmonary arterial hypertension (PAH) with right heart failure and active systemic lupus erythematosus who was effectively treated with an up-front triple combination of PAH therapies and immunosuppressive therapy. In case 2, a diagnosis of pulmonary veno-occlusive disease was reached after a combined approach of clinical suspicion, physical examination, and invasive and noninvasive tests...
December 2015: European Respiratory Review: An Official Journal of the European Respiratory Society
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