Pulmonary Hypertension in Systemic Sclerosis
TLDR
To select patients for this invasive procedure, several screening tools are discussed, including N-terminal pro-brain natriuretic peptide levels, uric acid levels, spirometry and diffusing capacity for carbon monoxide (DCLO), echocardiography (ECG), and the DETECT algorithm.Abstract:
The main cause of death in systemic sclerosis is interstitial lung disease, followed by pulmonary hypertension (PH). Pulmonary hypertension is the result of microvasculopathy which is caused by a disrupted healing process of endothelin damage and is featured by vasoconstriction, proliferation of arterial wall, inflammation, and fibrosis. Reclassification of pulmonary hypertension has led to five distinctive groups. In systemic sclerosis, patients may suffer from pulmonary artery hypertension (PAH, group 1), pulmonary hypertension due to interstitial lung disease (group 3), cardiac disease (group 2), and/ or thromboembolic pulmonary hypertension (group 4). Patients endure declining performance during exercise, but symptoms may be variable and nonspecific. Diagnosis is made by right heart catheterization. To select patients for this invasive procedure, several screening tools are discussed, including N-terminal pro-brain natriuretic peptide levels, uric acid levels, spirometry and diffusing capacity for carbon monoxide (DCLO), echocardiography (ECG), and the DETECT algorithm. Depending on features such as disease duration, presence of anti-centromere antibodies, and DCLO, three different flow charts for screening are presented. Based on pathophysiology, several medical treatments have been developed like prostanoids, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and stimulation of the nitric oxide pathway. Combination therapy as well as lung transplantation and supportive therapy is discussed.read more
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Journal ArticleDOI
2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT).
Nazzareno Galiè,Marc Humbert,Jean-Luc Vachiery,Simon Gibbs,Irene Lang,Adam Torbicki,Gérald Simonneau,Andrew Peacock,Anton Vonk Noordegraaf,Maurice Beghetti,Ardeschir Ghofrani,Miguel Angel Gomez Sanchez,Georg Hansmann,Walter Klepetko,Patrizio Lancellotti,Marco Matucci,Theresa McDonagh,Luc Pierard,Pedro T. Trindade,Maurizio Zompatori,Marius M. Hoeper +20 more
TL;DR: In this paper, the authors defined the following terms: ALAT, alanine aminotransferase, ASAT, aspartate AMINOTE, and APAH, associated pulmonary arterial hypertension.
Journal ArticleDOI
Updated clinical classification of pulmonary hypertension.
Gérald Simonneau,Michael A. Gatzoulis,Michael A. Gatzoulis,Ian Adatia,Ian Adatia,David S. Celermajer,David S. Celermajer,Christopher P. Denton,Ardeschir Ghofrani,Miguel Angel Gomez Sanchez,R. Krishna Kumar,Michael J. Landzberg,Roberto F. Machado,Horst Olschewski,Ivan M. Robbins,Rogiero Souza +15 more
TL;DR: In this paper, a clinical classification of pulmonary hypertension (PH) was established, categorizing PH into groups which share similar pathological and hemodynamic characteristics and therapeutic approaches, and the main change was to withdraw persistent pulmonary hypertension of the newborn (PPHN) from Group 1 because this entity carries more differences than similarities with other PAH subgroups.
Journal ArticleDOI
Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT)
Nazzareno Galiè,Marius M. Hoeper,Marc Humbert,Adam Torbicki,Jean-Luc Vachiery,J.A. Barberà,Maurice Beghetti,Paul A. Corris,Sean Gaine,J S R Gibbs,Miguel Ángel Gómez-Sánchez,Guillaume Jondeau,Walter Klepetko,Christian Opitz,Andrew J. Peacock,LJ Rubin,Michael J. Zellweger,Gérald Simonneau +17 more
TL;DR: In this paper, a review of the published evidence for management and/or prevention of a given condition is carried out by experts in the field and a critical evaluation of diagnostic and therapeutic procedures is performed including assessment of the risk/benefit ratio.
Journal ArticleDOI
Special article2015 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension
Nazzareno Galiè,Marc Humbert,Jean-Luc Vachiery,Simon Gibbs,Irene Lang,Adam Torbicki,Gérald Simonneau,Andrew J. Peacock,Anton Vonk Noordegraaf,Maurice Beghetti,Ardeschir Ghofrani,Miguel Angel Gomez Sanchez,Georg Hansmann,Walter Klepetko,Patrizio Lancellotti,Marco Matucci,Theresa McDonagh,Luc Pierard,Marius M. Hoeper +18 more
TL;DR: This article is being published concurrently in the European Heart Journal and the European Respiratory Journal and is identical except for minor stylistic and spelling differences in keeping with each journal’s style.
Journal ArticleDOI
Primary pulmonary hypertension: a national prospective study
Stuart Rich,D R Dantzker,S M Ayres,Edward H. Bergofsky,Bruce H. Brundage,Katherine M. Detre,Alfred P. Fishman,Roberta M. Goldring,Bertron M. Groves,Spencer K. Koerner +9 more
TL;DR: Although no deaths or sustained morbid events occurred during the diagnostic evaluation of the patients, the typically long interval from initial symptoms to diagnosis emphasizes the need to develop strategies to make the diagnosis earlier.