An Update on the Evaluation and Management of Pulmonary Hypertension in Scleroderma

被引：5

作者：

Coghlan, John G. ^{[1
]}

Schrieber, Benjamin ^{[2
]}

机构：

[1] Royal Free Hosp, Dept Cardiol, London NW3 2QG, England

[2] Royal Free Hosp, Dept Rheumatol, London NW3 2QG, England

来源：

CURRENT RHEUMATOLOGY REPORTS | 2012年 / 14卷 / 01期

关键词：

Systemic sclerosis; Scleroderma; Screening; Pulmonary hypertension; Pulmonary arterial hypertension; Goal-oriented therapy; Combination therapy; Endothelin receptor antagonist; Phosphodiesterase inhibitor; Prostanoid; Breathlessness; Pulmonary veno occlusive disease; PVOD; PAH; PH; ARTERIAL-HYPERTENSION; SYSTEMIC-SCLEROSIS; HEART-FAILURE; RECEPTOR ANTAGONIST; DOUBLE-BLIND; INTRAVENOUS EPOPROSTENOL; IMPROVED SURVIVAL; BOSENTAN; THERAPY; DISEASE;

D O I：

10.1007/s11926-011-0226-2

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Pulmonary arterial hypertension associated with scleroderma (SScPAH) is a debilitating, highly lethal condition that responds to an array of therapies. Quality of life and prognosis are substantially improved by treatment, and early diagnosis and treatment are associated with improved outcomes. There are serious limitations to current screening programs. Many more questions need to be addressed. Why is PAH so common in SSc? Why is the tolerance of pulmonary hypertension so poor in scleroderma? What are the best measures of response to therapy in SSc patients with PAH? Should we use different parameters in prognostic scores in SScPAH? Why is postcapillary pulmonary hypertension so common in SSc? How do we reliably differentiate lung disease-associated pulmonary hypertension from PAH? The aim of this review is to summarize the main areas of progress over the past decade and to look to the challenges for the next decade.

引用

页码：1 / 10

页数：10

共 61 条

[1] Long-term effects of darusentan on left-ventricular remodelling and clinical outcomes in the EndothelinA Receptor Antagonist Trial in Heart Failure (EARTH):: randomised, double-blind, placebo-controlled trial [J].

Anand, I ;

McMurray, J ;

Cohn, JN ;

Konstam, MA ;

Notter, T ;

Quitzau, K ;

Ruschitzka, F ;

Lüscher, TF .

LANCET, 2004, 364 (9431) :347-354

[2]

[Anonymous], 2002, Statistical Methods in Diagnostic Medicine

[3]

[Anonymous], EFF COMB BOS SILD VE

[4]

[Anonymous], 1998, WORLD S PRIM PULM HY

[5] Prevalence of Pulmonary Hypertension in Systemic Sclerosis in European Caucasians and Metaanalysis of 5 Studies [J].

Avouac, Jerome ;

Airo, Paulo ;

Meune, Christophe ;

Beretta, Lorenzo ;

Dieude, Philippe ;

Caramaschi, Paola ;

Tiev, Kiet ;

Cappelli, Susanna ;

Diot, Elisabeth ;

Vacca, Alessandra ;

Cracowski, Jean-Luc ;

Sibilia, Jean ;

Kahan, Andre ;

Matucci-Cerinic, Marco ;

Allanore, Yannick .

JOURNAL OF RHEUMATOLOGY, 2010, 37 (11) :2290-2298

[6] Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease -: A randomized, controlled trial [J].

Badesch, DB ;

Tapson, VF ;

McGoon, MD ;

Brundage, BH ;

Rubin, LJ ;

Wigley, FM ;

Rich, S ;

Barst, RJ ;

Barrett, PS ;

Kral, KM ;

Jöbsis, MM ;

Loyd, JE ;

Murali, S ;

Frost, A ;

Girgis, R ;

Bourge, RC ;

Ralph, DD ;

Elliott, CG ;

Hill, NS ;

Langleben, D ;

Schilz, RJ ;

McLaughlin, VV ;

Robbins, IM ;

Groves, BM ;

Shapiro, S ;

Medsger, TA ;

Gaine, SP ;

Horn, E ;

Decker, JC ;

Knobil, K .

ANNALS OF INTERNAL MEDICINE, 2000, 132 (06) :425-+

[7] Beraprost therapy for pulmonary arterial hypertension [J].

Barst, RJ ;

McGoon, M ;

McLaughlin, V ;

Tapson, V ;

Oudiz, R ;

Shapiro, S ;

Robbins, IM ;

Channick, R ;

Badesch, D ;

Rayburn, BK ;

Flinchbaugh, R ;

Sigman, J ;

Arneson, C ;

Jeffs, R .

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, 2003, 41 (12) :2119-2125

[8] Treatment of pulmonary arterial hypertension with the selective endothelin-A receptor antagonist sitaxsentan [J].

Barst, Robyn J. ;

Langleben, David ;

Badesch, David ;

Frost, Adaani ;

Lawrence, E. Clinton ;

Shapiro, Shelley ;

Naeije, Robert ;

Galie, Nazzareno .

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, 2006, 47 (10) :2049-2056

[9] Sitaxsentan for the Treatment of Pulmonary Arterial Hypertension A 1-Year, Prospective, Open-Label Observation of Outcome and Survival [J].

Benza, Raymond L. ;

Barst, Robyn J. ;

Galie, Nazzareno ;

Frost, Adaani ;

Girgis, Reda E. ;

Highland, Kristin B. ;

Strange, Charlie ;

Black, Carol M. ;

Badesch, David B. ;

Rubin, Lewis ;

Fleming, Thomas R. ;

Naeije, Robert .

CHEST, 2008, 134 (04) :775-782

[10] Predicting Survival in Pulmonary Arterial Hypertension Insights From the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) [J].

Benza, Raymond L. ;

Miller, Dave P. ;

Gomberg-Maitland, Mardi ;

Frantz, Robert P. ;

Foreman, Aimee J. ;

Coffey, Christopher S. ;

Frost, Adaani ;

Barst, Robyn J. ;

Badesch, David B. ;

Elliott, C. Gregory ;

Liou, Theodore G. ;

McGoon, Michael D. .

CIRCULATION, 2010, 122 (02) :164-U138

← 1 2 3 4 5 6 7 →