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Padte S, Mehta P, Bansal V, singh N, Sunasra R, Goyal V, Chaudhary RB, Junnarkar Y, Shah V, Arshad Z, Nawaz FA, Surani S, Kashyap R. Impact of diabetes mellitus on mortality in pulmonary hypertension: A systematic review and meta-analysis. World J Crit Care Med 2024; 13:99564. [PMID: 39655305 PMCID: PMC11577532 DOI: 10.5492/wjccm.v13.i4.99564] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/25/2024] [Revised: 09/05/2024] [Accepted: 09/23/2024] [Indexed: 10/31/2024] Open
Abstract
BACKGROUND Pulmonary hypertension (PH) is a progressive disease characterized by endothelial dysfunction and vascular remodeling and is a leading cause of mortality worldwide. Although it is independently associated with multiple comorbidities, the impact of diabetes mellitus (DM) on mortality in patients with PH remains uncertain. To address this issue, we conducted a systematic review and meta-analysis to investigate the effect of DM on survival in patients with pulmonary hypertension. AIM To investigate the impact of diabetes mellitus on mortality in pulmonary hypertension patients. METHODS We conducted a comprehensive search of four major electronic bibliographic databases like PubMed, Google Scholar, Scopus, and Embase, and identified 106 relevant studies, out of 1561 articles, published since the year 2000 for full-text review. Fourteen retrospective and prospective cohort studies that compared survival between patients with DM and those without DM in the context of PH were deemed eligible for inclusion in our meta-analysis. The study was registered on PROSPERO with the identifier CRD42023390232. RESULTS A total of 116455 patients with PH were included in the meta-analysis, of whom 41228 suffered from DM and 75227 did not. The results of our meta-analysis indicate an elevated mortality rate among PH patients with diabetes mellitus in comparison to those without DM [odds ratio (OR) = 1.40, 95%CI: 1.15-1.70, P = 0.0006]. The meta-regression analysis unveiled a statistically significant negative association between mean age and effect size (coefficient = -0.036, P value = 0.018). Conversely, a statistically significant positive association was detected between female proportion and effect size (coefficient = 0.000, P value < 0.001). CONCLUSION Our meta-analysis, which included approximately 116500 PH patients, revealed that the presence of diabetes mellitus was associated with increased odds of mortality when compared to non-diabetic patients. The meta-regression analysis indicates that studies with older participants and lower proportions of females tend to exhibit smaller effect sizes. Clinically, these findings underscore the importance of incorporating diabetes status into the risk stratification of patients with PH with more aggressive monitoring and early intervention to improve prognosis potentially.
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Affiliation(s)
- Smitesh Padte
- Department of Research, Global Remote Research Scholar Program, Princeton Junction, Princeton, NJ 08550, United States
- Department of Internal Medicine, WellSpan York Hospital, York, PA 17403, United States
| | - Priyal Mehta
- Department of Research, Global Remote Research Scholar Program, Princeton Junction, Princeton, NJ 08550, United States
- Department of Internal Medicine, St. Vincent Hospital, Worchester, MA 01608, United States
| | - Vikas Bansal
- Department of Nephrology and Hypertension, Mayo Clinic, Rochester, MN 55902, United States
| | - Niti singh
- Department of Anesthesiology and Critical Care, Seth G. S. Medical College and K.E.M. Hospital, Mumbai 400012, Mahārāshtra, India
| | - Rayyan Sunasra
- Department of Medicine, Hinduhridaysamrat Balasaheb Thackeray Medical College and Dr. R. N Cooper Hospital, Mumbai 400056, India
| | - Vidhi Goyal
- Department of Medicine, HBT Medical College and Dr. RN Cooper Hospital, Mumbai 400056, Mahārāshtra, India
| | - Raunaq B Chaudhary
- Department of Medicine, HBT Medical College and Dr. RN Cooper Hospital, Mumbai 400056, Mahārāshtra, India
| | - Yash Junnarkar
- Department of Medicine, HBT Medical College and Dr. RN Cooper Hospital, Mumbai 400056, Mahārāshtra, India
| | - Vidhi Shah
- Department of Medicine, HBT Medical College and Dr. RN Cooper Hospital, Mumbai 400056, Mahārāshtra, India
| | - Zara Arshad
- Department of Research, Global Remote Research Scholar Program, Princeton Junction, Princeton, NJ 08550, United States
| | - Faisal A Nawaz
- Department of Research, Global Remote Research Scholar Program, Princeton Junction, Princeton, NJ 08550, United States
- Department of Psychiatry, Al Amal Psychiatry Hospital, Dubai 50262, Dubayy, United Arab Emirates
| | - Salim Surani
- Department of Research, Global Remote Research Scholar Program, Princeton Junction, Princeton, NJ 08550, United States
- Department of Medicine & Pharmacology, Texas A&M University, College Station, TX 77843, United States
| | - Rahul Kashyap
- Department of Research, Global Remote Research Scholar Program, Princeton Junction, Princeton, NJ 08550, United States
- Department of Research, Wellspan Health, York, PA 17403, United States
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Cha JH, Jang SY, Song J, Kang IS, Huh J, Park TK, Yang JH, Park SW, Kim H, Kim DK, Chang SA. A Single Center Experience of Pulmonary Arterial Hypertension Management in Korea: A 25-Year Comparative Analysis Following the Introduction of Targeted Therapy. Korean Circ J 2024; 54:636-650. [PMID: 39175339 PMCID: PMC11522791 DOI: 10.4070/kcj.2023.0316] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/28/2023] [Revised: 04/27/2024] [Accepted: 06/04/2024] [Indexed: 08/24/2024] Open
Abstract
BACKGROUND AND OBJECTIVES The transformation of pulmonary arterial hypertension (PAH) treatment in Korea, ushered by targeted therapy's advent, prompted our analysis of baseline attributes, treatment trends, and survival shifts within our single-center registry. METHODS We examined 230 patients (72.6% female, mean age 40.6±17.4 years) diagnosed and/or treated between 1980 and 2021 in our PAH clinic. Given targeted therapy's introduction and active use since 2007, we compared diagnostic classification, demographics, and treatment patterns at that juncture. Survival analysis encompassed PAH types and the overall population. For historical survival comparison, 50 non-registry patients were retrospectively added, and age-sex matching enabled pooled analysis. RESULTS Congenital heart disease-associated pulmonary arterial hypertension (CHD-PAH) constituted the largest subset (43.0%), trailed by connective tissue disease-associated PAH (CTD-PAH, 29.6%) and idiopathic PAH (IPAH, 19.1%). Post-2007, CTD-PAH proportions surged, notably with an elevated initiation rate of targeted therapy (95.4%). Overall survival rates at 1, 5, and 10 years stood at 91.3%, 77.4%, and 65.8%, respectively, with CHD-PAH exhibiting superior survival to idiopathic or CTD-PAH. Age-sex matching analysis indicated survival disparities between those starting immediate targeted therapy vs. conservative treatment upon diagnosis, especially driven by IPAH. CONCLUSIONS In the post-introduction of the targeted therapy era, patients with PAH promptly started treatment right away, and higher survival rates of patients who started initial PAH-targeted therapy were demonstrated. The transition towards early treatment initiation might have likely contributed to the elevated survival rates observed in Korea's PAH patient cohort.
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Affiliation(s)
- Ji Hyun Cha
- Department of Critical Care Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
- Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
| | - Shin Yi Jang
- Pulmonary Hypertension Center, Heart Vascular Stroke Institute, Samsung Medical Center, Seoul, Korea
| | - Jinyoung Song
- Pulmonary Hypertension Center, Heart Vascular Stroke Institute, Samsung Medical Center, Seoul, Korea
- Division of Cardiology, Department of Pediatrics, Adult Congenital Heart Clinic, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
| | - I-Seok Kang
- Pulmonary Hypertension Center, Heart Vascular Stroke Institute, Samsung Medical Center, Seoul, Korea
- Division of Cardiology, Department of Pediatrics, Adult Congenital Heart Clinic, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
| | - June Huh
- Pulmonary Hypertension Center, Heart Vascular Stroke Institute, Samsung Medical Center, Seoul, Korea
- Division of Cardiology, Department of Pediatrics, Adult Congenital Heart Clinic, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
| | - Taek Kyu Park
- Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
- Pulmonary Hypertension Center, Heart Vascular Stroke Institute, Samsung Medical Center, Seoul, Korea
| | - Jeong Hoon Yang
- Department of Critical Care Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
- Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
- Pulmonary Hypertension Center, Heart Vascular Stroke Institute, Samsung Medical Center, Seoul, Korea
| | - Seung Woo Park
- Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
| | - Hojoong Kim
- Pulmonary Hypertension Center, Heart Vascular Stroke Institute, Samsung Medical Center, Seoul, Korea
- Division of Pulmonology and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
| | - Duk-Kyung Kim
- Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
- Pulmonary Hypertension Center, Heart Vascular Stroke Institute, Samsung Medical Center, Seoul, Korea
- Division of Cardiology, Department of Medicine, Samsung Changwon Hospital, Sungkyunkwan University School of Medicine, Changwon, Korea
| | - Sung-A Chang
- Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
- Pulmonary Hypertension Center, Heart Vascular Stroke Institute, Samsung Medical Center, Seoul, Korea.
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Mohammad M, Hartmann JP, Carlsen J, Greve AM, Berg RMG, Mortensen J. Prognostic value of pulmonary diffusing capacity for carbon monoxide and ventilation-perfusion SPECT findings in pulmonary arterial hypertension. Exp Physiol 2024; 109:1040-1050. [PMID: 38725160 PMCID: PMC11215485 DOI: 10.1113/ep091688] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/28/2023] [Accepted: 03/27/2024] [Indexed: 07/02/2024]
Abstract
Reduced pulmonary diffusing capacity for carbon monoxide (DLCO) can be observed in pulmonary arterial hypertension (PAH) and associates with increased mortality. However, the prognostic value of DLCO when corrected for haemoglobin (DLCOc), an independent modifier of DLCO, remains understudied. Additionally, the prognostic role of ventilation (V)-perfusion (Q) emission computed tomography (V/Q SPECT) findings in patients with PAH, which may concurrently be performed to rule out chronic thromboembolic pulmonary hypertension, is uncertain. A retrospective cohort study was conducted on 152 patients with PAH referred to a tertiary hospital for evaluation from January 2011 to January 2020. Lung function tests, clinical data and V/Q SPECT were ascertained. Cox regression analysis was performed to evaluate the association between DLCOc, DLCO and V/Q SPECT defects at referral with all-cause mortality. In equally adjusted Cox regression analysis, each percentage increase in DLCOc % predicted (%pred) (hazard ratio (HR) 0.97; 95% CI: 0.94-0.99) and DLCO%pred (HR 0.97; 95% CI: 0.94-0.99) was similarly associated with all-cause mortality. There was no detectable difference in area under the curve for prediction of all-cause mortality by DLCOc%pred and DLCO%pred (C-index 0.71 and 0.72, respectively, P = 0.85 for difference). None of the defects noted on V/Q SPECT were significantly associated with mortality, but mismatched defects were associated with lower values of DLCOc%pred and DLCO%pred. DLCOc%pred and DLCO%pred perform equally as prognostic markers in PAH, supporting the use of either metric when available for prognostic stratification.
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Affiliation(s)
- Milan Mohammad
- Centre for Physical Activity ResearchCopenhagen University Hospital – RigshospitaletCopenhagenDenmark
- Department of Biomedical Sciences, Faculty of Health and Medical SciencesUniversity of CopenhagenCopenhagenDenmark
| | - Jacob P. Hartmann
- Centre for Physical Activity ResearchCopenhagen University Hospital – RigshospitaletCopenhagenDenmark
- Department of Biomedical Sciences, Faculty of Health and Medical SciencesUniversity of CopenhagenCopenhagenDenmark
- Department of Clinical Physiology and Nuclear MedicineCopenhagen University Hospital – RigshospitaletCopenhagenDenmark
| | - Jørn Carlsen
- Department of CardiologyCopenhagen University Hospital RigshospitaletCopenhagenDenmark
- Department of Clinical Medicine, Faculty of Health and Medical SciencesUniversity of CopenhagenCopenhagenDenmark
| | - Anders M. Greve
- Department of Clinical BiochemistryCopenhagen University Hospital RigshospitaletCopenhagenDenmark
| | - Ronan M. G. Berg
- Centre for Physical Activity ResearchCopenhagen University Hospital – RigshospitaletCopenhagenDenmark
- Department of Biomedical Sciences, Faculty of Health and Medical SciencesUniversity of CopenhagenCopenhagenDenmark
- Department of Clinical Physiology and Nuclear MedicineCopenhagen University Hospital – RigshospitaletCopenhagenDenmark
- Neurovascular Research Laboratory, Faculty of Life Sciences and EducationUniversity of South WalesPontypriddUK
| | - Jann Mortensen
- Department of Clinical Physiology and Nuclear MedicineCopenhagen University Hospital – RigshospitaletCopenhagenDenmark
- Department of Clinical Medicine, Faculty of Health and Medical SciencesUniversity of CopenhagenCopenhagenDenmark
- Department of MedicineThe National HospitalTorshavnFaroe Islands
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Huang J, An Q, Shi H, Li C, Zhang W, Wang L. Retrospective cohort study of pulmonary arterial hypertension associated with connective tissue disease effect on patients' prognosis. Clin Rheumatol 2023; 42:3131-3142. [PMID: 37382842 DOI: 10.1007/s10067-023-06667-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/21/2023] [Revised: 06/05/2023] [Accepted: 06/07/2023] [Indexed: 06/30/2023]
Abstract
OBJECTIVE The objectives of this study are to clarify clinical characteristics and recognize prognostic factors of CTD-PAH patients. METHODS A retrospective cohort study of consecutive patients with documented CTD-PAH diagnosis from Jan 2014 to Dec 2019 was conducted, the ones who have other comorbid conditions that cause PH were excluded. Survival functions were plotted using the Kaplan-Meier method. Univariable and multivariable Cox regression analysis was applied to determine the survival-related factors. RESULTS In 144 patients with CTD-PAH analyzed, the median sPAP value was 52.5 (44.0, 71.0) mmHg, the overall targeted drug usage rate was 55.6%, and only 27.5% patients were given combination. Twenty-four non-PAH-CTD patients with sPAP value were included as the control group. Compared with non-PAH-CTD groups, CTD-PAH patients had worse cardiac function, higher NT-pro BNP and γ-globulin level, and lower PaCO2 level. Compared with the mild PAH group, the moderate-severe PAH group had worse cardiac function; increased Hb, HCT, and NP-pro BNP level; and decreased PaO2. Kaplan-Meier analysis showed significant difference for survival among non-PAH-CTD, mild CTD-PAH, and moderate-severe CTD-PAH groups. The univariate analyses showed that Hb, pH, and Ln (NT-pro BNP) were identified as factors significantly associated with survival, and Hb and pH showed significant association with risk of death in the multivariate model. Kaplan-Meier analysis also showed that Hb > 109.0 g/L and pH > 7.457 affected CTD-PAH patients' survival significantly. CONCLUSIONS PAH is not rare in CTDs patients; PAH affects CTD patients' prognosis significantly. Higher Hb and pH were associated with an increased risk of death. Key Points • Pulmonary arterial hypertension affects connective tissue disease patients' prognosis significantly. • The significantly factors associated with survival is hemoglobin, pH, and Ln (NT-pro BNP).
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Affiliation(s)
- Jing Huang
- Department of Rheumatism and Immunology, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, 710061, China
| | - Qi An
- Department of Rheumatism and Immunology, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, 710061, China
| | - Hongyang Shi
- Department of Respiratory and Critical Care Medicine, The Second Affiliated Hospital of Xi'an Jiaotong University (Xibei Hospital), No.157, Xiwu Road, Xincheng District, Xi'an, 710004, People's Republic of China
| | - Cong Li
- Department of Respiratory and Critical Care Medicine, The Second Affiliated Hospital of Xi'an Jiaotong University (Xibei Hospital), No.157, Xiwu Road, Xincheng District, Xi'an, 710004, People's Republic of China
| | - Wei Zhang
- Department of Emergency, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, 710061, China
| | - Lei Wang
- Department of Respiratory and Critical Care Medicine, The Second Affiliated Hospital of Xi'an Jiaotong University (Xibei Hospital), No.157, Xiwu Road, Xincheng District, Xi'an, 710004, People's Republic of China.
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Liu Y, Cheng Z, Zha B, Chen X, Gong Z, Ji L, Wei L. Risk factors of pulmonary arterial hypertension in patients with systemic lupus erythematosus: A meta-analysis. Lupus 2023; 32:1310-1319. [PMID: 37699157 DOI: 10.1177/09612033231202398] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/14/2023]
Abstract
OBJECTIVE To determine the risk factors of pulmonary arterial hypertension (PAH) related to systemic lupus erythematosus (SLE) through systematic reviews and meta-analyses. METHODS We undertook electronic search strategies using Medline via PubMed, Embase, Web of Science, and Cochrane Library up to April 11, 2023. Study selection and data extraction were performed by 2 authors independently. We made risk of bias judgments based on the Newcastle-Ottawa Scale (NOS). Pooled risk ratios (RRs) and 95% confidence intervals (CIs) were calculated to estimate the overall effect sizes of potential risk factors for PAH in SLE patients. Univariate and multivariate meta-regression models were used to assess the independent effects of each risk factor on PAH. Sensitivity analyses were also conducted to explore potential sources of heterogeneity. RESULTS A total of 19 articles were included in this meta-analysis, and the results showed that gender (female) [RR = 1.04, 95% CI (1.02, 1.06), p = .0001], interstitial lung disease [RR = 4.36, 95% CI (2.42, 7.85), p = .0001], alopecia [RR = 1.39, 95% CI (1.06, 1.83), p = .017], Raynaud's phenomenon [RR = 1.83, 95% CI (1.41, 2.37), p = .0001], systemic hypertension [RR = 1.30, 95% CI (1.07, 1.58), p = .007], serositis [RR = 2.29, 95% CI (1.89, 2.77), p = .0001], pericardial effusion [RR = 3.33, 95% CI (2.20, 5.05), p = .0001], anti-RNP [RR = 1.86, 95% CI (1.19, 2.91), p = .006], anti-SSA [RR = 1.28, 95% CI (1.01, 1.62), p = .041], anti-SSB [RR = 1.38, 95% CI (1.19, 1.60), p = .0001], anti-U1RNP [RR = 1.58, 95% CI (1.07, 2.34), p = .023], thrombocytopenia [RR = 1.38, 95% CI (1.14, 1.68), p = .001], and current smokers [RR = 2.20, 95% CI (1.19, 4.06), p = .012] were all risk factors for PAH related to SLE. CONCLUSION PAH is a serious complication of SLE. Since prognosis of SLE patients after the occurrence of PAH is poor, routine examination should be conducted for SLE patients with PAH risk factors.
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Affiliation(s)
- Yuqi Liu
- Beijing Luhe Hospital, Capital Medical University, Beijing, China
| | - Zhen Cheng
- Beijing Tiantan Hospital, Capital Medical University, Beijing, China
| | - Bowen Zha
- Beijing Anzhen Hospital, Capital Medical University, Beijing, China
| | - Xiaodong Chen
- Beijing Luhe Hospital, Capital Medical University, Beijing, China
| | - Zhiyu Gong
- Beijing Luhe Hospital, Capital Medical University, Beijing, China
| | - Lang Ji
- Central Laboratory, Beijing Luhe Clinical Institute, Capital Medical University, Beijing, China
| | - Lingling Wei
- Beijing Key Laboratory of Diabetic Prevention and Research, Center for Endocrine Metabolism and Immune Diseases, Beijing Luhe Hospital, Capital Medical University, Beijing, China
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Yang J, Zhou F, Zhou X, Sun Y, Lun X, Cao J, Fan B. Survival and prognosis analysis of systemic lupus erythematosus patients with pulmonary hypertension: A systematic review and meta-analysis. Medicine (Baltimore) 2023; 102:e34947. [PMID: 37682181 PMCID: PMC10489205 DOI: 10.1097/md.0000000000034947] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/13/2023] [Accepted: 08/04/2023] [Indexed: 09/09/2023] Open
Abstract
BACKGROUND The study aimed to evaluate survival rates and prognosis in systemic lupus erythematosus (SLE) patients with pulmonary hypertension (PH) using meta-analysis. METHODS PubMed, EMBASE, Cochrane central register of controlled trials, China National Knowledge Infrastructure, China Science and Technology Journal Database, Wan-Fang Database, and Chinese biomedical database were searched. Information and data were screened and extracted by 2 researchers. The obtained data were analyzed using the R software meta package. Quality assessment was conducted using Newcastle-Ottawa Scale. The causes of heterogeneity were analyzed using subgroup analysis and sensitivity analysis. Publication bias was evaluated using Begger funnel plots and Egger test. RESULTS The search strategy yielded a total of 21 studies involving 875 patients included in the final analysis. The pooled 1-, 3- and 5-year survival rates of patients with SLE-PH were 0.9020 (95%CI: 0.8576; 0.9397), 0.8363 (96%CI: 0.7813; 0.8852), 0.7301 (95%CI: 0.6327; 0.8181). The 1-, 3- and 5-year survival rates of echocardiography subgroup were 0.9000 (95%CI: 0.8480; 0.9551), 0.8435 (95%CI: 0.7744; 0.9187), 0.6795 (95%CI: 0.5746; 0.8035), respectively; and there were 0.9174 (95%CI: 0.8951; 0.9402), 0.8529 (95%CI: 0.8255; 0.8812), 0.7757 (95%CI: 0.7409; 0.8121) at right heart catheterization subgroup in the meantime. Multivariate analysis for predicting mortality in SLE-PH patients revealed that diminishing left ventricular ejection fraction, New York Heart Association classification, lupus nephritis, lower cardiac index, and higher red blood cell distribution width level were significantly associated with a higher mortality rate. Treatment with huge doses of cyclophosphamide, tricuspid annular plane systolic excursion/pulmonary artery systolic pressure, and Raynaud phenomenon signaled favorable outcomes. CONCLUSION The 1-, 3-, and 5-year survival rates of SLE-PH patients in recent years (0.9020, 0.8363, 0.7301) were estimated in this study. SLE-PH patients diagnosed by echocardiography have a worse long-term prognosis than those diagnosed by right heart catheterization. Studies after 2015 have shown significantly better survival than earlier studies.
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Affiliation(s)
- Jianguo Yang
- Shandong University of Traditional Chinese Medicine, Jinan, Shandong, People’s Republic of China
| | - Fuyu Zhou
- Shandong University of Traditional Chinese Medicine, Jinan, Shandong, People’s Republic of China
| | - Xinpeng Zhou
- Affiliated Hospital of Shandong University of Traditional Chinese Medicine, Jinan, Shandong, People’s Republic of China
| | - Yuying Sun
- Shandong University of Traditional Chinese Medicine, Jinan, Shandong, People’s Republic of China
| | - Xueping Lun
- Shandong University of Traditional Chinese Medicine, Jinan, Shandong, People’s Republic of China
| | - Jiaojiao Cao
- Shandong University of Traditional Chinese Medicine, Jinan, Shandong, People’s Republic of China
| | - Bing Fan
- Affiliated Hospital of Shandong University of Traditional Chinese Medicine, Jinan, Shandong, People’s Republic of China
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Shin JI, Lee KH, Park S, Yang JW, Kim HJ, Song K, Lee S, Na H, Jang YJ, Nam JY, Kim S, Lee C, Hong C, Kim C, Kim M, Choi U, Seo J, Jin H, Yi B, Jeong SJ, Sheok YO, Kim H, Lee S, Lee S, Jeong YS, Park SJ, Kim JH, Kronbichler A. Systemic Lupus Erythematosus and Lung Involvement: A Comprehensive Review. J Clin Med 2022; 11:jcm11226714. [PMID: 36431192 PMCID: PMC9698564 DOI: 10.3390/jcm11226714] [Citation(s) in RCA: 27] [Impact Index Per Article: 9.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/12/2022] [Revised: 10/27/2022] [Accepted: 11/04/2022] [Indexed: 11/16/2022] Open
Abstract
Systemic lupus erythematosus (SLE) is a complex autoimmune disease with multiorgan manifestations, including pleuropulmonary involvement (20-90%). The precise mechanism of pleuropulmonary involvement in SLE is not well-understood; however, systemic type 1 interferons, circulating immune complexes, and neutrophils seem to play essential roles. There are eight types of pleuropulmonary involvement: lupus pleuritis, pleural effusion, acute lupus pneumonitis, shrinking lung syndrome, interstitial lung disease, diffuse alveolar hemorrhage (DAH), pulmonary arterial hypertension, and pulmonary embolism. DAH has a high mortality rate (68-75%). The diagnostic tools for pleuropulmonary involvement in SLE include chest X-ray (CXR), computed tomography (CT), pulmonary function tests (PFT), bronchoalveolar lavage, biopsy, technetium-99m hexamethylprophylene amine oxime perfusion scan, and (18)F-fluorodeoxyglucose positron emission tomography. An approach for detecting pleuropulmonary involvement in SLE includes high-resolution CT, CXR, and PFT. Little is known about specific therapies for pleuropulmonary involvement in SLE. However, immunosuppressive therapies such as corticosteroids and cyclophosphamide are generally used. Rituximab has also been successfully used in three of the eight pleuropulmonary involvement forms: lupus pleuritis, acute lupus pneumonitis, and shrinking lung syndrome. Pleuropulmonary manifestations are part of the clinical criteria for SLE diagnosis. However, no review article has focused on the involvement of pleuropulmonary disease in SLE. Therefore, this article summarizes the literature on the epidemiology, pathogenesis, diagnosis, and management of pleuropulmonary involvement in SLE.
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Affiliation(s)
- Jae Il Shin
- Department of Pediatrics, Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Keum Hwa Lee
- Department of Pediatrics, Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Seoyeon Park
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Jae Won Yang
- Department of Nephrology, Yonsei University Wonju College of Medicine, Wonju 26426, Republic of Korea
| | - Hyung Ju Kim
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Kwanhyuk Song
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Seungyeon Lee
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Hyeyoung Na
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Yong Jun Jang
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Ju Yun Nam
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Soojin Kim
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Chaehyun Lee
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Chanhee Hong
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Chohwan Kim
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Minhyuk Kim
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Uichang Choi
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Jaeho Seo
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Hyunsoo Jin
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - BoMi Yi
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Se Jin Jeong
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Yeon Ook Sheok
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Haedong Kim
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Sangmin Lee
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Sangwon Lee
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Young Soo Jeong
- Yonsei University College of Medicine, Seoul 03722, Republic of Korea
| | - Se Jin Park
- Department of Pediatrics, Eulji University School of Medicine, Daejeon 34824, Republic of Korea
| | - Ji Hong Kim
- Department of Pediatrics, Yonsei University College of Medicine, Seoul 03722, Republic of Korea
- Department of Pediatrics, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul 26426, Republic of Korea
- Correspondence:
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Zhao J, Wang Q, Deng X, Qian J, Tian Z, Liu Y, Li M, Zeng X. The treatment strategy of connective tissue disease associated pulmonary arterial hypertension: Evolving into the future. Pharmacol Ther 2022; 239:108192. [DOI: 10.1016/j.pharmthera.2022.108192] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/31/2021] [Revised: 04/07/2022] [Accepted: 04/18/2022] [Indexed: 11/30/2022]
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9
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Huang J, An Q, Zhang CL, He L, Wang L. Decreased low‑density lipoprotein and the presence of pulmonary arterial hypertension among newly diagnosed drug‑naïve patients with systemic lupus erythematosus: D‑dimer as a mediator. Exp Ther Med 2022; 24:595. [PMID: 35949327 PMCID: PMC9353521 DOI: 10.3892/etm.2022.11531] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/14/2022] [Accepted: 06/13/2022] [Indexed: 11/23/2022] Open
Abstract
Pulmonary arterial hypertension (PAH) is commonly associated with systemic lupus erythematosus (SLE). The present study investigated the relationship between coagulation and changes in lipid parameters in newly-diagnosed patients with SLE in the presence of PAH and whether the coagulation parameters were mediators between lipids and PAH presence. A total of 301 subjects scheduled for new-onset drug-naïve SLE were consecutively enrolled. Baseline data for patients without PAH and with PAH were gathered and compared. Coagulation and lipid parameters were compared across patients without lipid regulating and anticoagulation medications. Multivariable logistic regression model was applied to examine potential predictors of PAH in SLE. The relationships between them were examined using Spearman's correlation analysis. The relationship between coagulation index and lipids with SLE-PAH was evaluated using mediation analysis. Female patients accounted for 88.0% of the 301 subjects, and the average age was 32 years (range, 25-45 years). A total of 40 patients (13.3%) had PAH, and the average pulmonary artery systolic pressure (sPAP) was 55.825±26.67 mmHg. Patients with PAH were older and had higher levels of fibrin/fibrinogen degradation products (FDP), D-dimer, C-reactive protein, lower levels of complement 3, complement 4 and 25-hydroxy vitamin D3 compared with the non-PAH group. Multivariable logistic regression analysis showed that age and D-dimer were independent predictor factors for PAH. Among patients without lipid regulating and anticoagulation medications, patients in the PAH group had higher levels of D-dimer and FDP, and lower low-density lipoprotein (LDL) levels compared with patients without PAH. There was also a positive relationship between sPAP and D-dimer and FDP, and a negative relationship between sPAP and total cholesterol and LDL. Mediation analysis indicated that 25.61% of the effect of low LDL on PAH presence in systemic lupus erythematosus was mediated by D-dimer. Overall, the effect of low LDL on SLE-PAH appeared to be mediated by D-dimer, which mediated 25.61% of this effect.
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Affiliation(s)
- Jing Huang
- Department of Rheumatism and Immunology, First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi 710061, P.R. China
| | - Qi An
- Department of Rheumatism and Immunology, First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi 710061, P.R. China
| | - Cai-Lian Zhang
- Department of Pulmonary and Critical Care Medicine, Yan'an University Affiliated Hospital, Yan'an, Shaanxi 716000, P.R. China
| | - Lan He
- Department of Rheumatism and Immunology, First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi 710061, P.R. China
| | - Lei Wang
- Department of Pulmonary and Critical Care Medicine, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi 710004, P.R. China
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10
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Wang RR, Yuan TY, Wang JM, Chen YC, Zhao JL, Li MT, Fang LH, Du GH. Immunity and inflammation in pulmonary arterial hypertension: From pathophysiology mechanisms to treatment perspective. Pharmacol Res 2022; 180:106238. [DOI: 10.1016/j.phrs.2022.106238] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/06/2022] [Revised: 04/27/2022] [Accepted: 04/27/2022] [Indexed: 02/08/2023]
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11
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Diamanti E, Karava V, Yerly P, Aubert JD. Carbon Monoxide Diffusion Capacity as a Severity Marker in Pulmonary Hypertension. J Clin Med 2021; 11:jcm11010132. [PMID: 35011871 PMCID: PMC8745155 DOI: 10.3390/jcm11010132] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/26/2021] [Revised: 12/22/2021] [Accepted: 12/23/2021] [Indexed: 01/09/2023] Open
Abstract
Carbon monoxide diffusion capacity (DLCO) is negatively associated with patient survival in idiopathic pulmonary hypertension (PH), but is not included in the risk stratification score proposed by the 2015 European guidelines. Since 2015, several new stratification scores based on a 3- or 4-severity scale have been explored. This retrospective cohort single-center study sought to investigate the association between DLCO and PH severity and survival. We included 85 treatment-naive patients with precapillary PH and DLCO measurement at diagnosis. DLCO status, based on lower and upper quartiles ranges, was added to a 3- and a 4-strata modified-risk assessment. DLCO was strongly associated with transplant-free survival (HR 0.939, 95% CI: 0.908–0.971, p < 0.001). In the intermediate and high-risk categories, DLCO was associated with transplant-free survival, irrespective of the risk category (HR 0.934, 95% CI: 0.880–0.980, p = 0.005). The correlation between modified-risk category and transplant-free survival was significant (HR 4.60, 95% CI: 1.294–16.352, p = 0.018). Based on the Akaike information criterion (AIC) levels, the 3- and 4-strata modified-risk stratification fits our results better than the conventional stratification. Low DLCO is associated with patient transplant-free survival, independently of the risk category. Inclusion of DLCO into a PH risk stratification score seems promising and needs further investigation.
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Affiliation(s)
- Eleni Diamanti
- Division of Pulmonology, Lausanne University Hospital, University of Lausanne, CH-1011 Lausanne, Switzerland;
| | - Vasiliki Karava
- 1st Department of Pediatrics, Aristotle University of Thessaloniki, 541 24 Thessaloniki, Greece;
| | - Patrick Yerly
- Division of Cardiology, Lausanne University Hospital, University of Lausanne, CH-1011 Lausanne, Switzerland;
| | - John David Aubert
- Division of Pulmonology, Lausanne University Hospital, University of Lausanne, CH-1011 Lausanne, Switzerland;
- Correspondence:
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12
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Pan J, Lei L, Zhao C, Wen J, Qin F, Dong F. Clinical characteristics and survival of patients with three major connective tissue diseases associated with pulmonary hypertension: A study from China. Exp Ther Med 2021; 22:925. [PMID: 34306194 PMCID: PMC8280713 DOI: 10.3892/etm.2021.10357] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/04/2020] [Accepted: 03/17/2021] [Indexed: 02/06/2023] Open
Abstract
The present cross-sectional study investigated the clinical characteristics and survival of patients with three types of connective tissue disease associated with pulmonary hypertension (CTD-PH) diagnosed early by echocardiography. A total of 218 patients with CTD-PH were included in the present study. Patients with the three major types of CTD, namely systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and primary Sjögren's syndrome (pSS), were included. PH was diagnosed based on pulmonary arterial systolic pressure >35 mmHg, as measured by Doppler echocardiography. Demographic data, clinical features, laboratory results and echocardiographic parameters were collected and analyzed. The Kaplan-Meier method was used to calculate survival rates. Multivariate analysis was used to identify independent factors affecting mortality. Compared with patients with CTD with pSS (6.5%) or SLE (3.8%), those with SSc had a higher prevalance of PH (12.9%). Patients with SSc-PH had the highest rate of lung involvement (81.2%) and 42.2% of patients were classified as World Health Organization-function class III/IV at the time of diagnosis with PH. The overall survival rate among patients with CTD-PH at 1, 3 and 5 years was 81.4, 72.4 and 56.9%, respectively. Patients with SLE-PH appeared to have the most favorable prognosis and patients with SSc-PH had the poorest relative outcomes. Multivariate analysis revealed that age ≥50 years was the only independent risk factor for mortality. In conclusion, among the patients with CTDs investigated, the prevalence of PH was highest among those with SSc. Patients with SSc-PH had the highest prevalence of pulmonary involvement, the lowest survival rate and the worst prognosis.
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Affiliation(s)
- Jie Pan
- Department of Rheumatology and Immunology, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi Zhuang Autonomous Region 530000, P.R. China
| | - Ling Lei
- Department of Rheumatology and Immunology, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi Zhuang Autonomous Region 530000, P.R. China
| | - Cheng Zhao
- Department of Rheumatology and Immunology, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi Zhuang Autonomous Region 530000, P.R. China
| | - Jing Wen
- Department of Rheumatology and Immunology, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi Zhuang Autonomous Region 530000, P.R. China
| | - Fang Qin
- Department of Rheumatology and Immunology, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi Zhuang Autonomous Region 530000, P.R. China
| | - Fei Dong
- Department of Rheumatology and Immunology, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi Zhuang Autonomous Region 530000, P.R. China
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13
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Qu J, Li M, Wang Y, Duan X, Luo H, Zhao C, Zhan F, Wu Z, Li H, Yang M, Xu J, Wei W, Wu L, Liu Y, You H, Qian J, Yang X, Huang C, Zhao J, Wang Q, Leng X, Tian X, Zhao Y, Zeng X. Predicting the Risk of Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus: A Chinese Systemic Lupus Erythematosus Treatment and Research Group Cohort Study. Arthritis Rheumatol 2021; 73:1847-1855. [PMID: 34105259 DOI: 10.1002/art.41740] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/22/2020] [Accepted: 03/16/2021] [Indexed: 12/17/2022]
Abstract
OBJECTIVE Pulmonary arterial hypertension (PAH) is a life-threatening complication of systemic lupus erythematosus (SLE). However, there is no algorithm to identify those at high risk. This study was undertaken to develop a prediction model for PAH in patients with lupus that provides individualized risk estimates. METHODS A multicenter, longitudinal cohort study was undertaken from January 2003 to January 2020. The study collected data on 3,624 consecutively evaluated patients diagnosed as having SLE. The diagnosis of PAH was confirmed by right-sided heart catheterization. Cox proportional hazards regression and least absolute shrinkage and selection operator were used to fit the model. Model discrimination, calibration, and decision curve analysis were performed for validation. RESULTS Ninety-two lupus patients (2.54%) developed PAH during a median follow-up of 4.84 years (interquartile range 2.42-8.84). The final prediction model included 5 clinical variables (acute/subacute cutaneous lupus, arthritis, renal disorder, thrombocytopenia, and interstitial lung disease) and 3 autoantibodies (anti-RNP, anti-Ro/SSA and anti-La/SSB). A 10-year PAH probability-predictive nomogram was established. The model was internally validated by Harrell's concordance index (0.78), the Brier score (0.03), and a satisfactory calibration curve. According to the net benefit and predicted probability thresholds, we recommend annual screening in high-risk (>4.62%) lupus patients. CONCLUSION We developed a risk stratification model using routine clinical assessments. This new tool may effectively predict the future risk of PAH in patients with SLE.
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Affiliation(s)
- Jingge Qu
- Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, and Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Mengtao Li
- Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, and Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Yanhong Wang
- Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Xinwang Duan
- Second Affiliated Hospital of Nanchang University, Nanchang, China
| | - Hui Luo
- Xiangya Hospital and Central South University, Changsha, China
| | - Cheng Zhao
- First Affiliated Hospital of Guangxi Medical University, Nanning, China
| | - Feng Zhan
- Hainan General Hospital and Hainan Affiliated Hospital of Hainan Medical University, Haikou, China
| | - Zhenbiao Wu
- Xijing First Affiliated Hospital of the Fourth Military Medical University, Xi'an, China
| | - Hongbin Li
- Affiliated Hospital of Inner Mongolia Medical College, Hohhot, China
| | - Min Yang
- Nanfang Hospital and Southern Medical University, Guangzhou, China
| | - Jian Xu
- First Affiliated Hospital of Kunming Medical University, Kunming, China
| | - Wei Wei
- Tianjin Medical University General Hospital, Tianjin, China
| | - Lijun Wu
- People's Hospital of Xinjiang Uygur Autonomous Region, Urumchi, China
| | - Yongtai Liu
- Peking Union Medical College, Chinese Academy of Medical Sciences and Peking Union Medical College Hospital, Beijing, China
| | - Hanxiao You
- Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, and Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Juyan Qian
- Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, and Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Xiaoxi Yang
- Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, and Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Can Huang
- Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, and Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Jiuliang Zhao
- Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, and Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Qian Wang
- Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, and Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Xiaomei Leng
- Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, and Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Xinping Tian
- Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, and Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Yan Zhao
- Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, and Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Xiaofeng Zeng
- Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, and Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
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14
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Khanna D, Zhao C, Saggar R, Mathai SC, Chung L, Coghlan JG, Shah M, Hartney J, McLaughlin V. Long-Term Outcomes in Patients With Connective Tissue Disease-Associated Pulmonary Arterial Hypertension in the Modern Treatment Era: Meta-Analyses of Randomized, Controlled Trials and Observational Registries. Arthritis Rheumatol 2021; 73:837-847. [PMID: 33538058 PMCID: PMC8251834 DOI: 10.1002/art.41669] [Citation(s) in RCA: 35] [Impact Index Per Article: 8.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/18/2020] [Accepted: 01/26/2021] [Indexed: 12/13/2022]
Abstract
Objective Data on the magnitude of benefit of modern therapies for pulmonary arterial hypertension (PAH) in connective tissue disease (CTD)–associated PAH are limited. In this study, we performed meta‐analyses of randomized, controlled trials (RCTs) and registries to quantify the benefit of these modern therapies in patients with CTD‐PAH. Methods The PubMed and Embase databases were searched for articles reporting data from RCTs or registries published between January 1, 2000 and November 25, 2019. Eligibility criteria included multicenter studies with ≥30 CTD‐PAH patients. For an RCT to be included, the trial had to evaluate an approved PAH therapy, and long‐term risks of clinical morbidity and mortality or 6‐minute walk distance had to be reported. For a registry to be included, survival rates had to be reported. Random‐effects models were used to pool the data. Results Eleven RCTs (total of 4,329 patients; 1,267 with CTD‐PAH) and 19 registries (total of 9,739 patients; 4,008 with CTD‐PAH) were included. Investigational therapy resulted in a 36% reduction in the risk of clinical morbidity/mortality events both in the overall PAH population (hazard ratio [HR] 0.64, 95% confidence interval [95% CI] 0.54, 0.75; P < 0.001) and in CTD‐PAH patients (HR 0.64, 95% CI 0.51, 0.81; P < 0.001) as compared to control subjects. The survival rate was lower in CTD‐PAH patients compared to all PAH patients (survival rate 62%, 95% CI 57, 67% versus 72%, 95% CI 69, 75% at 3 years). The survival rate in CTD‐PAH patients treated primarily after 2010 was higher than that in CTD‐PAH patients treated before 2010 (survival rate 73%, 95% CI 62, 81% versus 65%, 95% CI 59, 71% at 3 years). Conclusion Modern therapy provides a similar reduction in morbidity/mortality risk in patients with CTD‐PAH when compared to the PAH population overall. Risk of death is higher in CTD‐PAH patients than in those with PAH overall, but survival has improved in the last 10 years, which may be related to increased screening and/or new treatment approaches. Early detection of PAH in patients with CTD and up‐front intensive treatment are warranted.
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Affiliation(s)
| | - Carol Zhao
- Actelion Pharmaceuticals US, Inc., South San Francisco, California
| | | | - Stephen C Mathai
- Johns Hopkins University School of Medicine, Baltimore, Maryland
| | | | | | - Mehul Shah
- Actelion Pharmaceuticals US, Inc., South San Francisco, California
| | - John Hartney
- Actelion Pharmaceuticals US, Inc., South San Francisco, California
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15
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Seo MR, Yeo J, Ryu HJ, Choi HJ, Ko KP, Baek HJ. Outcomes and Risk Factors of Systolic Pulmonary Artery Pressure Progression in Patients with Systemic Rheumatic Diseases: Follow-up Results from a Korean Registry. Arch Rheumatol 2021; 35:558-567. [PMID: 33758812 PMCID: PMC7945697 DOI: 10.46497/archrheumatol.2020.7812] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2019] [Accepted: 12/02/2019] [Indexed: 11/20/2022] Open
Abstract
Objectives
This study aims to investigate the outcomes and risk factors associated with the progression of systolic pulmonary artery pressure (sPAP) in patients with systemic rheumatic diseases. Patients and methods
A total of 532 patients (73 males, 459 females; median age 49 years; interquartile range (IQR), 36 to 62 years) registered with the Registry of Pulmonary Hypertension Associated with Rheumatic Diseases were included. Mortality curves were constructed using the Kaplan- Meier method and comparisons were performed using the log-rank test. A paired t-test was performed to evaluate the patients with markedly elevated sPAP between baseline and follow-up. Results
The average follow-up duration was 31 months (IQR, 9 to 60 months). Of the patients, 196 had follow-up echocardiographs at least one year later. We defined the sPAP over 60 mmHg as markedly elevated. Patients in the increased sPAP above 60 mmHg at follow-up and persistently markedly elevated sPAP were associated with worse outcomes in all-cause mortality and pulmonary arterial hypertension-related mortality (p<0.001). In patients with systemic sclerosis, the majority of patients remained static within their pressure group or rose progressively: the patients with markedly elevated sPAP at follow-up were higher than those at baseline (32% versus 15%, p<0.01). In patients with mixed connective tissue disease (MCTD) or rheumatoid arthritis (RA), the majority of patients remained static within their pressure group or gradually improved: the patients with markedly elevated sPAP at follow-up were lower than those at baseline (RA=14% versus 29%, MCTD=5% versus 16%, p<0.05). Conclusion Persistently high sPAP or increase of sPAP over 60 mmHg at follow-up was associated with increased mortality. There were some differences in the progression of sPAP according to the underlying rheumatic diseases.
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Affiliation(s)
- Mi Ryoung Seo
- Department of Internal Medicine, Division of Rheumatology, Gil Medical Center, Gachon University College of Medicine, Incheon, South Korea
| | - Jina Yeo
- Department of Internal Medicine, Division of Rheumatology, Gil Medical Center, Gachon University College of Medicine, Incheon, South Korea
| | - Hee Jung Ryu
- Department of Internal Medicine, Division of Rheumatology, Gil Medical Center, Gachon University College of Medicine, Incheon, South Korea
| | - Hyo-Jin Choi
- Department of Internal Medicine, Division of Rheumatology, Gil Medical Center, Gachon University College of Medicine, Incheon, South Korea
| | - Kwang-Pil Ko
- Department of Preventive Medicine, Gil Medical Center, Gachon University College of Medicine, Incheon, South Korea
| | - Han Joo Baek
- Department of Internal Medicine, Division of Rheumatology, Gil Medical Center, Gachon University College of Medicine, Incheon, South Korea
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Sonaglioni A, Cassandro R, Luisi F, Ferrante D, Nicolosi GL, Lombardo M, Anzà C, Harari S. Correlation Between Doppler Echocardiography and Right Heart Catheterisation-Derived Systolic and Mean Pulmonary Artery Pressures: Determinants of Discrepancies Between the Two Methods. Heart Lung Circ 2020; 30:656-664. [PMID: 33223493 DOI: 10.1016/j.hlc.2020.10.009] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/29/2020] [Revised: 08/27/2020] [Accepted: 10/06/2020] [Indexed: 01/28/2023]
Abstract
BACKGROUND There is still controversy about whether transthoracic echocardiography (TTE) can provide reliable estimations of pulmonary artery pressures (PAP). The primary endpoint of this study was to evaluate the correlation between TTE and right heart catheterisation (RHC) in estimating systolic (SPAP) and mean (MPAP) pulmonary artery pressures. METHODS Between January 2011 and December 2018, 141 consecutive patients (average age 63.6±11.5 years; 84 women) with suspected or confirmed pulmonary hypertension (PH) were enrolled into this retrospective observational monocentric study. All patients underwent TTE and, within 3 hours, RHC. The correlation between TTE and RHC in estimating both SPAP and MPAP was retrospectively determined. RESULTS Seventeen (17) of the patients were excluded due to insufficient TTE signal quality. Of the remaining 124 patients, 18 had no PH. There was moderate correlation between both SPAP and MPAP estimated by TTE and those assessed by RHC (r=0.65 and r=0.60, respectively). Bland-Altman analysis revealed a bias of -11.9 mmHg (with the 95% limits of agreement ranging -45.4 to +21.5 mmHg) for SPAP estimation and -4.6 mmHg (with the 95% limits of agreement ranging -27.9 to +18.8 mmHg) for MPAP estimation, suggesting a general overestimation of PAP by TTE. The main factors responsible for discrepancies between TTE and RHC were: female gender, arrhythmic cardiac electrical activity, systemic arterial hypertension, and diuretic treatment. CONCLUSIONS Transthoracic echocardiography frequently overestimated PAP in comparison with RHC, especially in hypertensive women with arrhythmias and under diuretic treatment.
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Affiliation(s)
- Andrea Sonaglioni
- Department of Cardiology, Ospedale San Giuseppe MultiMedica IRCCS, Milan, Italy
| | - Roberto Cassandro
- Department of Pneumology, Semi-Intensive Care Unit, Department of Respiratory Physiopathology and Pulmonary Hemodynamics, Ospedale San Giuseppe MultiMedica IRCCS, Milan, Italy.
| | - Francesca Luisi
- Department of Pneumology, Semi-Intensive Care Unit, Department of Respiratory Physiopathology and Pulmonary Hemodynamics, Ospedale San Giuseppe MultiMedica IRCCS, Milan, Italy
| | - Daniela Ferrante
- Unit of Medical Statistics and Epidemiology, CPO Piemonte and University 'Amedeo Avogadro' of Piemonte Orientale, Novara, Italy
| | | | - Michele Lombardo
- Department of Cardiology, Ospedale San Giuseppe MultiMedica IRCCS, Milan, Italy
| | - Claudio Anzà
- Cardiovascular Department, MultiMedica IRCCS, Sesto San Giovanni (MI), Italy
| | - Sergio Harari
- Department of Pneumology, Semi-Intensive Care Unit, Department of Respiratory Physiopathology and Pulmonary Hemodynamics, Ospedale San Giuseppe MultiMedica IRCCS, Milan, Italy; Department of Medical Sciences San Giuseppe Hospital MultiMedica IRCCS and Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy
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Lin CY, Ko CH, Hsu CY, Chen HA. Epidemiology and mortality of connective tissue disease-associated pulmonary arterial hypertension: A national cohort study in taiwan. Semin Arthritis Rheum 2020; 50:957-962. [DOI: 10.1016/j.semarthrit.2020.06.005] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/01/2019] [Revised: 05/13/2020] [Accepted: 06/09/2020] [Indexed: 12/22/2022]
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18
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Fox RI. The incidence of pulmonary hypertension is higher in systemic lupus and Sjögren's patients than in scleroderma patients in China. Lupus 2020; 27:1051-1052. [PMID: 29732959 DOI: 10.1177/0961203318772019] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/17/2022]
Affiliation(s)
- R I Fox
- Scripps Memorial Hospital and Research Institute La Jolla, CA, USA
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19
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Stadler S, Mergenthaler N, Lange TJ. The prognostic value of DLCO and pulmonary blood flow in patients with pulmonary hypertension. Pulm Circ 2019; 9:2045894019894531. [PMID: 31908765 PMCID: PMC6935895 DOI: 10.1177/2045894019894531] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/01/2019] [Accepted: 11/19/2019] [Indexed: 12/12/2022] Open
Abstract
Background Cardiac output is a prognostic marker in patients with pulmonary hypertension. Pulmonary blood flow as a surrogate for cardiac output can be measured non-invasively by inert gas rebreathing. We hypothesized that pulmonary blood flow can predict outcome in patients with pulmonary hypertension. Methods From January 2009 to January 2012, we measured pulmonary blood flow by inert gas rebreathing in outpatients with pulmonary hypertension. Patients with pulmonary hypertension confirmed by right heart catheterization and a valid inert gas rebreathing maneuver were followed until January 2016. The investigated outcome was all-cause mortality. Results We included 259 patients (mean age 65 ± 13 years, 53% female) with pulmonary hypertension and classified into groups 1 (n = 103), 2 (n = 26), 3 (n = 80), and 4 (n = 50) according to the current pulmonary hypertension classification system. The median time between pulmonary hypertension diagnosis and inert gas rebreathing was 9 (IQR 0; 36) months. During a median follow-up time of 51 (IQR 20; 68) months, 109 patients (42%) died. Parameters significantly associated with survival (in order of decreasing statistical strength) were diffusion capacity of the lung for carbon monoxide (DLCO), 6-minute walk distance (6-MWD), age, NTpro-BNP, WHO functional class, group 3 pulmonary hypertension, and tricuspid annular plane systolic excursion (TAPSE), while baseline hemodynamics and pulmonary blood flow were not. In multivariable Cox regression analysis, DLCO, age, 6-MWD, and TAPSE remained significant and independent predictors of the outcome. DLCO as the strongest parameter also significantly predicted survival in aetiological subgroups except for group 4. Conclusions DLCO is a strong and independent predictor for survival in patients with pulmonary hypertension of different aetiologies, while pulmonary blood flow measured by inert gas rebreathing is not.
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Affiliation(s)
- Stefan Stadler
- Department of Internal Medicine II, University Medical Center Regensburg, Regensburg, Germany
| | - Nicoletta Mergenthaler
- Department of Internal Medicine II, University Medical Center Regensburg, Regensburg, Germany
| | - Tobias J Lange
- Department of Internal Medicine II, University Medical Center Regensburg, Regensburg, Germany
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Chen HA, Hsu TC, Yang SC, Weng CT, Wu CH, Sun CY, Lin CY. Incidence and survival impact of pulmonary arterial hypertension among patients with systemic lupus erythematosus: a nationwide cohort study. Arthritis Res Ther 2019; 21:82. [PMID: 30917868 PMCID: PMC6438012 DOI: 10.1186/s13075-019-1868-0] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/15/2018] [Accepted: 03/18/2019] [Indexed: 12/31/2022] Open
Abstract
Background No population-based study has investigated the cumulative incidence of pulmonary arterial hypertension (PAH) in patients with newly diagnosed systemic lupus erythematosus (SLE) or the survival impact of PAH in this population. Method We used a nationwide database in Taiwan and enrolled incident SLE patients between January 1, 2000, and December 31, 2013. The cumulative incidence of PAH in the SLE patients and the survival of these patients were estimated by the Kaplan-Meier method. Potential predictors of the development of PAH were determined using a Cox proportional hazards regression model. Results Of 15,783 SLE patients, 336 (2.13%) developed PAH. The average interval from SLE diagnosis to PAH diagnosis was 3.66 years (standard deviation [SD] 3.36, range 0.1 to 13.0 years). Seventy percent of the patients developed PAH within 5 years after SLE onset. The 3- and 5-year cumulative incidence of PAH were 1.2% and 1.8%, respectively. Systemic hypertension was an independent predictor of PAH occurrence among the SLE patients (adjusted hazard ratio 2.27, 95% confidence interval 1.59–2.97). The 1-, 3-, and 5-year survival rates of SLE patients following the diagnosis of PAH were 87.7%, 76.8%, and 70.1%, respectively. Conclusions PAH is a rare complication of SLE and the majority of PAH cases occur within the first 5 years following SLE diagnosis. Systemic hypertension may be a risk factor for PAH development in the SLE population. The overall 5-year survival rate after PAH diagnosis was 70.1%.
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Affiliation(s)
- Hung-An Chen
- Division of Allergy-Immunology-Rheumatology, Department of Internal Medicine, Chi Mei Medical Center, Tainan, Taiwan.,Chia Nan University of Pharmacy and Science, Tainan, Taiwan
| | - Tsai-Ching Hsu
- Institute of Biochemistry, Microbiology and Immunology, Chung Shan Medical University, Taichung, Taiwan
| | - Su-Ching Yang
- Department of Nursing, National Tainan Institute of Nursing, Tainan, Taiwan
| | - Chia-Tse Weng
- Department of Internal Medicine, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, No.138, Sheng Li Road, Tainan, 704, Taiwan
| | - Chun-Hsin Wu
- Department of Internal Medicine, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, No.138, Sheng Li Road, Tainan, 704, Taiwan
| | - Chien-Yao Sun
- Department of Internal Medicine, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, No.138, Sheng Li Road, Tainan, 704, Taiwan
| | - Chun-Yu Lin
- Department of Internal Medicine, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, No.138, Sheng Li Road, Tainan, 704, Taiwan.
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21
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Zhang N, Li M, Qian J, Wang Q, Zhao J, Yang Z, Tian Z, Zhang X, Zuo X, Zhang M, Zhu P, Ye S, Zhang W, Zheng Y, Qi W, Li Y, Zhang Z, Ding F, Gu J, Liu Y, Wei W, Zeng X. Pulmonary arterial hypertension in systemic lupus erythematosus based on a CSTAR-PAH study: Baseline characteristics and risk factors. Int J Rheum Dis 2019; 22:921-928. [PMID: 30746850 DOI: 10.1111/1756-185x.13478] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/15/2018] [Revised: 12/09/2018] [Accepted: 12/17/2018] [Indexed: 02/05/2023]
Abstract
AIM Pulmonary arterial hypertension (PAH) is a complex and devastating complication of systemic lupus erythematosus (SLE). We sought to describe the baseline characteristics of right heart catheterization (RHC)-confirmed SLE-associated PAH and identify risk factors for PAH in SLE patients. METHODS A multicenter, cross-sectional study was conducted using the Chinese SLE Treatment and Research group (CSTAR) registry. Baseline data for patients with SLE-associated PAH and SLE patients without PAH were collected and compared. Risk factors for PAH among patients with SLE were identified. RESULTS A total of 292 patients with SLE-associated PAH were enrolled. RHC was used to reveal hemodynamic features, including mean pulmonary arterial pressure (46.2 ± 12.0 mm Hg), pulmonary arterial wedge pressure (7.84 ± 3.92 mm Hg), pulmonary vascular resistance (10.86 ± 5.57 Wood units), and cardiac index (2.77 ± 0.91 L/min × m2 ). A multivariate logistic regression analysis showed that serositis (odds ratio [OR] = 5.524, 95% CI 3.605-8.465, P < 0.001), anti-ribonucleoprotein (RNP) antibody positivity (OR = 13.332, 95% CI 9.500-18.710, P < 0.001), and diffusion capacity of carbon monoxide in the lung (DLCO)/%Pred <70% (OR = 10.018, 95% CI 6.619-15.162, P < 0.001) were independent predictors of PAH. We recommend using transthoracic echocardiography (TTE) to perform early screening of SLE patients who have serositis, anti-RNP antibody positivity, or DLCO/%Pred <70%. RHC is suggested for patients suspected of having PAH. Once a diagnosis of SLE-PAH is confirmed, evaluation and treatment should immediately begin. CONCLUSION Overall, we recommend performing early screening using TTE in SLE patients with serositis, anti-RNP antibodies, or a DLCO/%Pred <70%, even for patients in a relatively stable condition according to SLE disease activity index.
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Affiliation(s)
- Na Zhang
- Department of Rheumatology, Tianjin Medical University General Hospital, Tianjin, China
| | - Mengtao Li
- Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.,Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Junyan Qian
- Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.,Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Qian Wang
- Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.,Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Jiuliang Zhao
- Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.,Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
| | - Zhenwen Yang
- Department of Cardiology, Tianjin Medical University General Hospital, Tianjin, China
| | - Zhuang Tian
- Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - Xiao Zhang
- Department of Rheumatology, Guangdong General Hospital, Guangzhou, China
| | - Xiaoxia Zuo
- Department of Rheumatology, Xiangya Hospital, Central South University, Changsha, China
| | - Miaojia Zhang
- Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, China
| | - Ping Zhu
- Department of Clinical Immunology, PLA Specialized Research Institute of Rheumatology & Immunology, Xijing Hospital, The Fourth Military Medical University, Xi'an, China
| | - Shuang Ye
- Department of Rheumatology, Ren Ji Hospital South Campus, School of Medicine, Shanghai Jiao Tong University, Shanghai, China
| | - Wei Zhang
- Department of Rheumatology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China
| | - Yi Zheng
- Department of Rheumatology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China
| | - Wufang Qi
- Department of Rheumatology, The First Central Hospital, Tianjin, China
| | - Yang Li
- Department of Rheumatology, The Second Affiliated Hospital of Harbin Medical University, Harbin, China
| | - Zhuoli Zhang
- Department of Rheumatology and Clinical Immunology, Peking University First Hospital, Beijing, China
| | - Feng Ding
- Department of Rheumatology, Qilu Hospital of Shandong University, Jinan, China
| | - Jieruo Gu
- Department of Rheumatology, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China
| | - Yi Liu
- Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, China
| | - Wei Wei
- Department of Rheumatology, Tianjin Medical University General Hospital, Tianjin, China
| | - Xiaofeng Zeng
- Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.,Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
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22
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Jung JY, Lee CH, Kim HA, Choi ST, Lee JH, Yoon BY, Kang DR, Suh CH. Pulmonary Hypertension in Connective Tissue Disease is Associated with the New York Heart Association Functional Class and Forced Vital Capacity, But Not with Interstitial Lung Disease. JOURNAL OF RHEUMATIC DISEASES 2018; 25:179. [DOI: 10.4078/jrd.2018.25.3.179] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/13/2017] [Revised: 03/14/2018] [Accepted: 03/28/2018] [Indexed: 08/30/2023]
Affiliation(s)
- Ju-Yang Jung
- Department of Rheumatology, Ajou University School of Medicine, Suwon, Korea
| | - Chan Hee Lee
- Department of Rheumatology, National Health Insurance Service Ilsan Hospital, Goyang, Korea
| | - Hyoun-Ah Kim
- Department of Rheumatology, Ajou University School of Medicine, Suwon, Korea
| | - Sang Tae Choi
- Department of Rheumatology, Chung-Ang University College of Medicine, Seoul, Korea
| | - Joo-Hyun Lee
- Division of Rheumatology, Department of Internal Medicine, Inje University Ilsan Paik Hospital, Goyang, Korea
| | - Bo-Young Yoon
- Division of Rheumatology, Department of Internal Medicine, Inje University Ilsan Paik Hospital, Goyang, Korea
| | - Dae-Ryong Kang
- Department of Medical Humanities and Social Medicine, Office of Biostatistics, Ajou University School of Medicine, Suwon, Korea
| | - Chang-Hee Suh
- Department of Rheumatology, Ajou University School of Medicine, Suwon, Korea
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23
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Li Y, Wang Y, Li H, Zhu W, Meng X, Lu X. Evaluation of the hemodynamics and right ventricular function in pulmonary hypertension by echocardiography compared with right-sided heart catheterization. Exp Ther Med 2017; 14:3616-3622. [PMID: 29042956 PMCID: PMC5639404 DOI: 10.3892/etm.2017.4953] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/12/2016] [Accepted: 03/17/2017] [Indexed: 11/24/2022] Open
Abstract
The present study aimed to evaluate hemodynamics and right ventricular function in patients with pulmonary hypertension (PH) using transthoracic echocardiography and to compare these results with measurements obtained using right-sided heart catheterization (RHC). A total of 75 patients with PH were examined using echocardiography and RHC. Patients were divided into the following two groups according to their difference between SPAPecho and SPAPRHC measurement: The overestimated group and underestimated group. The overestimated group included the subgroups groupover-A (difference <20 mmHg) and groupover-B (difference ≥20 mmHg), and the underestimated group included groupunder-A (absolute value of the difference <20 mmHg) and groupunder-B (absolute value of the difference ≥20 mmHg). SPAPecho measurements were revealed to be significantly positively correlated with SPAPRHC measurements (r=0.794; P<0.01). Among all echocardiographic measurements, only tricuspid annular plane systolic excursion (TAPSE) was significantly different between groups; it was increased in groupover-A and groupunder-A compared with groupover-B (P<0.01). Although SPAP measurements obtained using echocardiography were significantly positively correlated with those obtained using RHC, a high proportion of overestimation or underestimation of SPAP by echocardiography remained.
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Affiliation(s)
- Yidan Li
- Department of Echocardiography, Heart Center, Beijing Chao Yang Hospital, Capital Medical University, Beijing 100020, P.R. China
| | - Yidan Wang
- Department of Echocardiography, Heart Center, Beijing Chao Yang Hospital, Capital Medical University, Beijing 100020, P.R. China
| | - Hong Li
- Department of Echocardiography, Heart Center, Beijing Chao Yang Hospital, Capital Medical University, Beijing 100020, P.R. China
| | - Weiwei Zhu
- Department of Echocardiography, Heart Center, Beijing Chao Yang Hospital, Capital Medical University, Beijing 100020, P.R. China
| | - Xiangli Meng
- Department of Echocardiography, Heart Center, Beijing Chao Yang Hospital, Capital Medical University, Beijing 100020, P.R. China
| | - Xiuzhang Lu
- Department of Echocardiography, Heart Center, Beijing Chao Yang Hospital, Capital Medical University, Beijing 100020, P.R. China
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24
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van Riel ACMJ, Opotowsky AR, Santos M, Rivero JM, Dhimitri A, Mulder BJM, Bouma BJ, Landzberg MJ, Waxman AB, Systrom DM, Shah AM. Accuracy of Echocardiography to Estimate Pulmonary Artery Pressures With Exercise: A Simultaneous Invasive-Noninvasive Comparison. Circ Cardiovasc Imaging 2017; 10:CIRCIMAGING.116.005711. [PMID: 28360262 DOI: 10.1161/circimaging.116.005711] [Citation(s) in RCA: 65] [Impact Index Per Article: 8.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/21/2016] [Accepted: 02/06/2017] [Indexed: 01/15/2023]
Abstract
BACKGROUND Exercise echocardiography is often applied as a noninvasive strategy to screen for abnormal pulmonary hemodynamic response, but it is technically challenging, and limited data exist regarding its accuracy to estimate pulmonary arterial pressure during exercise. METHODS AND RESULTS Among 65 patients with exertional intolerance undergoing upright invasive exercise testing, tricuspid regurgitation (TR) Doppler estimates and invasive measurement of pulmonary arterial pressure at rest and peak exercise were simultaneously obtained. TR Doppler envelopes were assessed for quality. Correlation, Bland-Altman, and receiver-operating characteristic curve analyses were performed to evaluate agreement and diagnostic accuracy. Mean age was 62±13 years, and 31% were male. High-quality (grade A) TR Doppler was present in 68% at rest and 34% at peak exercise. For grade A TR signals, echocardiographic measures of systolic pulmonary arterial pressure correlated reasonably well with invasive measurement at rest (r=0.72, P<0.001; bias, -2.9±8.0 mm Hg) and peak exercise (r=0.75, P<0.001; bias, -1.9±15.6 mm Hg). Lower quality TR signals (grade B and C) correlated poorly with invasive measurements overall. In patients with grade A TR signals, mean pulmonary arterial pressure-to-workload ratio at a threshold of 1.4 mm Hg/10 W was able to identify abnormal pulmonary hemodynamic response during exercise (>3.0 mm Hg/L per minute increase), with 91% sensitivity and 82% specificity (area under the curve, 0.90; 95% confidence interval, 0.77-1.0; P=0.001). CONCLUSIONS Agreement between echocardiographic and invasive measures of pulmonary pressures during upright exercise is good among the subset of patients with high-quality TR Doppler signal. While the limits of agreement are broad, our results suggest that in those patients, sensitivity is adequate to screen for abnormal pulmonary hemodynamic response during exercise.
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Affiliation(s)
- Annelieke C M J van Riel
- From the Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands (A.C.M.J.v.R., B.J.M.M., B.J.B.); Netherlands Heart Institute, Utrecht (A.C.M.J.v.R., B.J.M.M.); Department of Cardiology, Boston Children's Hospital, and Harvard Medical School, MA (A.R.O., M.J.L.); Cardiovascular Medicine, Department of Medicine (A.R.O., J.M.R., A.D., M.J.L., A.M.S.) and Pulmonary and Critical Care Medicine, Department of Medicine, (A.B.W., D.M.S.), Brigham and Women's Hospital and Harvard Medical School, Boston, MA; and Department of Physiology and Cardiothoracic Surgery, Cardiovascular R&D Unit, Faculty of Medicine, University of Porto, Portugal (M.S.)
| | - Alexander R Opotowsky
- From the Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands (A.C.M.J.v.R., B.J.M.M., B.J.B.); Netherlands Heart Institute, Utrecht (A.C.M.J.v.R., B.J.M.M.); Department of Cardiology, Boston Children's Hospital, and Harvard Medical School, MA (A.R.O., M.J.L.); Cardiovascular Medicine, Department of Medicine (A.R.O., J.M.R., A.D., M.J.L., A.M.S.) and Pulmonary and Critical Care Medicine, Department of Medicine, (A.B.W., D.M.S.), Brigham and Women's Hospital and Harvard Medical School, Boston, MA; and Department of Physiology and Cardiothoracic Surgery, Cardiovascular R&D Unit, Faculty of Medicine, University of Porto, Portugal (M.S.)
| | - Mário Santos
- From the Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands (A.C.M.J.v.R., B.J.M.M., B.J.B.); Netherlands Heart Institute, Utrecht (A.C.M.J.v.R., B.J.M.M.); Department of Cardiology, Boston Children's Hospital, and Harvard Medical School, MA (A.R.O., M.J.L.); Cardiovascular Medicine, Department of Medicine (A.R.O., J.M.R., A.D., M.J.L., A.M.S.) and Pulmonary and Critical Care Medicine, Department of Medicine, (A.B.W., D.M.S.), Brigham and Women's Hospital and Harvard Medical School, Boston, MA; and Department of Physiology and Cardiothoracic Surgery, Cardiovascular R&D Unit, Faculty of Medicine, University of Porto, Portugal (M.S.)
| | - Jose M Rivero
- From the Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands (A.C.M.J.v.R., B.J.M.M., B.J.B.); Netherlands Heart Institute, Utrecht (A.C.M.J.v.R., B.J.M.M.); Department of Cardiology, Boston Children's Hospital, and Harvard Medical School, MA (A.R.O., M.J.L.); Cardiovascular Medicine, Department of Medicine (A.R.O., J.M.R., A.D., M.J.L., A.M.S.) and Pulmonary and Critical Care Medicine, Department of Medicine, (A.B.W., D.M.S.), Brigham and Women's Hospital and Harvard Medical School, Boston, MA; and Department of Physiology and Cardiothoracic Surgery, Cardiovascular R&D Unit, Faculty of Medicine, University of Porto, Portugal (M.S.)
| | - Andy Dhimitri
- From the Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands (A.C.M.J.v.R., B.J.M.M., B.J.B.); Netherlands Heart Institute, Utrecht (A.C.M.J.v.R., B.J.M.M.); Department of Cardiology, Boston Children's Hospital, and Harvard Medical School, MA (A.R.O., M.J.L.); Cardiovascular Medicine, Department of Medicine (A.R.O., J.M.R., A.D., M.J.L., A.M.S.) and Pulmonary and Critical Care Medicine, Department of Medicine, (A.B.W., D.M.S.), Brigham and Women's Hospital and Harvard Medical School, Boston, MA; and Department of Physiology and Cardiothoracic Surgery, Cardiovascular R&D Unit, Faculty of Medicine, University of Porto, Portugal (M.S.)
| | - Barbara J M Mulder
- From the Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands (A.C.M.J.v.R., B.J.M.M., B.J.B.); Netherlands Heart Institute, Utrecht (A.C.M.J.v.R., B.J.M.M.); Department of Cardiology, Boston Children's Hospital, and Harvard Medical School, MA (A.R.O., M.J.L.); Cardiovascular Medicine, Department of Medicine (A.R.O., J.M.R., A.D., M.J.L., A.M.S.) and Pulmonary and Critical Care Medicine, Department of Medicine, (A.B.W., D.M.S.), Brigham and Women's Hospital and Harvard Medical School, Boston, MA; and Department of Physiology and Cardiothoracic Surgery, Cardiovascular R&D Unit, Faculty of Medicine, University of Porto, Portugal (M.S.)
| | - Berto J Bouma
- From the Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands (A.C.M.J.v.R., B.J.M.M., B.J.B.); Netherlands Heart Institute, Utrecht (A.C.M.J.v.R., B.J.M.M.); Department of Cardiology, Boston Children's Hospital, and Harvard Medical School, MA (A.R.O., M.J.L.); Cardiovascular Medicine, Department of Medicine (A.R.O., J.M.R., A.D., M.J.L., A.M.S.) and Pulmonary and Critical Care Medicine, Department of Medicine, (A.B.W., D.M.S.), Brigham and Women's Hospital and Harvard Medical School, Boston, MA; and Department of Physiology and Cardiothoracic Surgery, Cardiovascular R&D Unit, Faculty of Medicine, University of Porto, Portugal (M.S.)
| | - Michael J Landzberg
- From the Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands (A.C.M.J.v.R., B.J.M.M., B.J.B.); Netherlands Heart Institute, Utrecht (A.C.M.J.v.R., B.J.M.M.); Department of Cardiology, Boston Children's Hospital, and Harvard Medical School, MA (A.R.O., M.J.L.); Cardiovascular Medicine, Department of Medicine (A.R.O., J.M.R., A.D., M.J.L., A.M.S.) and Pulmonary and Critical Care Medicine, Department of Medicine, (A.B.W., D.M.S.), Brigham and Women's Hospital and Harvard Medical School, Boston, MA; and Department of Physiology and Cardiothoracic Surgery, Cardiovascular R&D Unit, Faculty of Medicine, University of Porto, Portugal (M.S.)
| | - Aaron B Waxman
- From the Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands (A.C.M.J.v.R., B.J.M.M., B.J.B.); Netherlands Heart Institute, Utrecht (A.C.M.J.v.R., B.J.M.M.); Department of Cardiology, Boston Children's Hospital, and Harvard Medical School, MA (A.R.O., M.J.L.); Cardiovascular Medicine, Department of Medicine (A.R.O., J.M.R., A.D., M.J.L., A.M.S.) and Pulmonary and Critical Care Medicine, Department of Medicine, (A.B.W., D.M.S.), Brigham and Women's Hospital and Harvard Medical School, Boston, MA; and Department of Physiology and Cardiothoracic Surgery, Cardiovascular R&D Unit, Faculty of Medicine, University of Porto, Portugal (M.S.)
| | - David M Systrom
- From the Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands (A.C.M.J.v.R., B.J.M.M., B.J.B.); Netherlands Heart Institute, Utrecht (A.C.M.J.v.R., B.J.M.M.); Department of Cardiology, Boston Children's Hospital, and Harvard Medical School, MA (A.R.O., M.J.L.); Cardiovascular Medicine, Department of Medicine (A.R.O., J.M.R., A.D., M.J.L., A.M.S.) and Pulmonary and Critical Care Medicine, Department of Medicine, (A.B.W., D.M.S.), Brigham and Women's Hospital and Harvard Medical School, Boston, MA; and Department of Physiology and Cardiothoracic Surgery, Cardiovascular R&D Unit, Faculty of Medicine, University of Porto, Portugal (M.S.)
| | - Amil M Shah
- From the Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands (A.C.M.J.v.R., B.J.M.M., B.J.B.); Netherlands Heart Institute, Utrecht (A.C.M.J.v.R., B.J.M.M.); Department of Cardiology, Boston Children's Hospital, and Harvard Medical School, MA (A.R.O., M.J.L.); Cardiovascular Medicine, Department of Medicine (A.R.O., J.M.R., A.D., M.J.L., A.M.S.) and Pulmonary and Critical Care Medicine, Department of Medicine, (A.B.W., D.M.S.), Brigham and Women's Hospital and Harvard Medical School, Boston, MA; and Department of Physiology and Cardiothoracic Surgery, Cardiovascular R&D Unit, Faculty of Medicine, University of Porto, Portugal (M.S.).
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25
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Tselios K, Gladman DD, Urowitz MB. Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies. Open Access Rheumatol 2016; 9:1-9. [PMID: 28053559 PMCID: PMC5191623 DOI: 10.2147/oarrr.s123549] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022] Open
Abstract
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. Its pathogenesis is multifactorial with vasoconstriction, due to imbalance of vasoactive mediators, leading to hypoxia and impaired vascular remodeling, collagen deposition, and thrombosis of the pulmonary circulation. Multiple predictive factors have been recognized, such as Raynaud’s phenomenon, pleuritis, pericarditis, anti-ribonuclear protein, and antiphospholipid antibodies. Secure diagnosis is based on right heart catheterization, although transthoracic echocardiogram has been shown to be reliable for patient screening and follow-up. Data on treatment mostly come from uncontrolled observational studies and consist of immunosuppressive drugs, mainly corticosteroids and cyclophosphamide, as well as PAH-targeted approaches with endothelin receptor antagonists (bosentan), phosphodiesterase type 5 inhibitors (sildenafil), and vasodilators (epoprostenol). Prognosis is significantly affected, with 1- and 5-year survival estimated at 88% and 68%, respectively.
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Affiliation(s)
- Konstantinos Tselios
- University of Toronto Lupus Clinic, Centre for Prognosis Studies in the Rheumatic Diseases, University Health Network, Toronto, ON, Canada
| | - Dafna D Gladman
- University of Toronto Lupus Clinic, Centre for Prognosis Studies in the Rheumatic Diseases, University Health Network, Toronto, ON, Canada
| | - Murray B Urowitz
- University of Toronto Lupus Clinic, Centre for Prognosis Studies in the Rheumatic Diseases, University Health Network, Toronto, ON, Canada
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