Recommendations for screening and detection of connective tissue disease-associated pulmonary arterial hypertension.
Recommendations for screening and detection of connective tissue disease-associated pulmonary arterial hypertension.
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DOI：
10.1002/art.38172
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发表时间：
2013-12
影响因子：
--
通讯作者：
McLaughlin, Vallerie V.
中科院分区：
文献类型：
--
作者：
Khanna, Dinesh;Gladue, Heather;Channick, Richard;Chung, Lorinda;Distler, Oliver;Furst, Daniel E.;Hachulla, Eric;Humbert, Marc;Langleben, David;Mathai, Stephen C.;Saggar, Rajeev;Visovatti, Scott;Altorok, Nezam;Townsend, Whitney;FitzGerald, John;McLaughlin, Vallerie V.
肺动脉高压（PAH）影响高达15%的结缔组织病（CTD）患者。作为PAH更大努力的一部分而制定的先前建议没有为CTD-PAH患者提供详细建议。因此，我们寻求制定CTD-PAH筛查和早期检测的建议。我们通过检索文献对CTD中PAH的筛查和诊断进行了系统综述。使用兰德公司/加州大学洛杉矶分校的方法，我们开发的情况下，其次是2个阶段的投票-第一，来自不同专业的国际专家匿名投票的适当性，每个情况下的情况下，然后专家举行了面对面的会议，讨论和解决不一致的投票，以达成共识的建议。关键建议指出，系统性硬化症（SSc）患者应接受PAH筛查。此外，混合性结缔组织病（MCTD）或其他CTD与硬皮病的特点，也应筛选PAH（硬皮病谱系障碍）。SSc和硬皮病谱系疾病患者的初步筛查评估包括肺功能测试（PFT）（包括一氧化碳弥散量（DLCO））、经胸超声心动图（TTE）和NT-Pro BNP。在SSc和谱系障碍中，应每年进行TTE和PFT。一旦出现任何新的体征或症状，应立即进行全面筛查（TTE、PFT和NT-ProBNP）。我们为CTD-PAH的筛查和早期检测提供基于共识的循证建议。我们希望这些建议将导致CTD-PAH的早期检测，并最终改善患者的预后。
Pulmonary arterial hypertension (PAH) affects up to 15% of patients with connective tissue diseases (CTD). Previous recommendations developed as part of larger efforts in PAH did not provide detailed recommendations for patients with CTD-PAH. Therefore, we sought to develop recommendations for screening and early detection of CTD-PAH. We performed a systematic review for the screening and diagnosis of PAH in CTD by searching the literature. Using the RAND/UCLA methodology, we developed case scenarios followed by 2 stages of voting—first international experts from a variety of specialties voted anonymously on the appropriateness of each case scenario and then the experts met in a face-to-face meeting to discuss and resolve discrepant votes to arrive at consensus recommendations. The key recommendations state that patients with systemic sclerosis (SSc) should be screened for PAH. In addition, mixed connective tissue diseases (MCTD) or other CTD’s with scleroderma features should also be screened for PAH (scleroderma-spectrum disorder). Initial screening evaluation in patients with SSc and scleroderma-spectrum disorders include pulmonary function test (PFT) including diffusion capacity carbon monoxide (DLCO), transthoracic echocardiogram (TTE), and NT- Pro BNP. In SSc and spectrum disorders, TTE and PFT should be performed on annual basis. The full screening panel (TTE, PFT, and NT-ProBNP) should be performed as soon as any new signs or symptoms are present. We provide consensus-based, evidence-driven recommendations for screening and early detection of CTD-PAH. It is our hope that these recommendations will lead to earlier detection of CTD-PAH and ultimately improve patient outcomes.
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影响因子：
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作者：
Allanore, Y.;Avouac, J.;Kahan, A.
通讯作者：
Kahan, A.
影响因子：
4.7
作者：
Khanna, Dinesh;Khanna, Puja P.;Fitzgerald, John D.;Singh, Manjit K.;Bae, Sangmee;Neogi, Tuhina;Pillinger, Michael H.;Merill, Joan;Lee, Susan;Prakash, Shraddha;Kaldas, Marian;Gogia, Maneesh;Perez-Ruiz, Fernando;Taylor, Will;Liote, Frederic;Choi, Hyon;Singh, Jasvinder A.;Dalbeth, Nicola;Kaplan, Sanford;Niyyar, Vandana;Jones, Danielle;Yarows, Steven A.;Roessler, Blake;Kerr, Gail;King, Charles;Levy, Gerald;Furst, Daniel E.;Edwards, N. Lawrence;Mandell, Brian;Schumacher, H. Ralph;Robbins, Mark;Wenger, Neil;Terkeltaub, Robert
通讯作者：
Terkeltaub, Robert
影响因子：
4.7
作者：
Khanna, Dinesh;Fitzgerald, John D.;Khanna, Puja P.;Bae, Sangmee;Singh, Manjit K.;Neogi, Tuhina;Pillinger, Michael H.;Merill, Joan;Lee, Susan;Prakash, Shraddha;Kaldas, Marian;Gogia, Maneesh;Perez-Ruiz, Fernando;Taylor, Will;Liote, Frederic;Choi, Hyon;Singh, Jasvinder A.;Dalbeth, Nicola;Kaplan, Sanford;Niyyar, Vandana;Jones, Danielle;Yarows, Steven A.;Roessler, Blake;Kerr, Gail;King, Charles;Levy, Gerald;Furst, Daniel E.;Edwards, N. Lawrence;Mandell, Brian;Schumacher, H. Ralph;Robbins, Mark;Wenger, Neil;Terkeltaub, Robert
通讯作者：
Terkeltaub, Robert
影响因子：
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作者：
Humbert, Marc;Yaici, Azzedine;Hachulla, Eric
通讯作者：
Hachulla, Eric
影响因子：
24
作者：
Badesch, David B.;Champion, Hunter C.;Torbicki, Adam
通讯作者：
Torbicki, Adam