Pulmonary Arterial Hypertension In Systemic Sclerosis: Challenges In Diagnosis, Screening And Treatment

被引：20

作者：

Saygin, Didem ^{[1
]}

Domsic, Robyn T. ^{[2
]}

机构：

[1] Univ Pittsburgh, Med Ctr, Dept Med, Pittsburgh, PA 15213 USA

[2] Univ Pittsburgh, Sch Med, Div Rheumatol & Clin Immunol, S706 BST,200 Lothrop St, Pittsburgh, PA 15213 USA

来源：

OPEN ACCESS RHEUMATOLOGY-RESEARCH AND REVIEWS | 2019年 / 11卷

关键词：

scleroderma; pulmonary hypertension; screening; CONNECTIVE-TISSUE DISEASE; COMBINATION THERAPY; SUBGROUP ANALYSIS; RISK-FACTORS; BOSENTAN; SURVIVAL; AMBRISENTAN; PREDICTORS; ECHOCARDIOGRAPHY; MONOTHERAPY;

D O I：

10.2147/OARRR.S228234

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Systemic sclerosis (SSc) is a chronic, multisystem autoimmune disease characterized by vasculopathy, fibrosis and immune system activation. Pulmonary hypertension and interstitial lung disease account for majority of SSc-related deaths. Diagnosis of SSc-PAH can be challenging due to nonspecific clinical presentation which can lead to delayed diagnosis. Many screening algorithms have been developed to detect SSc-associated pulmonary arterial hypertension (SSc-PAH) in early stages. Currently used PAH-specific medications are largely extrapolated from IPAH studies due to smaller number of patients with SSc-PAH. In this review, we discuss the current state of knowledge in epidemiology and risk factors for development of SSc-PAH, and challenges and potential solutions in the diagnosis, screening and management of SSc-PAH.

引用

页码：323 / 333

页数：11

共 64 条

[1] High N-terminal pro-brain natriuretic peptide levels and low diffusing capacity for carbon monoxide as independent predictors of the occurrence of precapillary pulmonary arterial hypertension in patients with systemic sclerosis [J].

Allanore, Y. ;

Avouac, J. ;

Zerkak, D. ;

Meune, C. ;

Hachulla, E. ;

Mouthon, L. ;

Guillevin, L. ;

Meyer, O. ;

Ekindjian, O. G. ;

Weber, S. ;

Kahan, A. .

ARTHRITIS AND RHEUMATISM, 2008, 58 (01) :284-291

[2] Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease [J].

Arcasoy, SM ;

Christie, JD ;

Ferrari, VA ;

Sutton, MS ;

Zisman, DA ;

Blumenthal, NP ;

Pochettino, A ;

Kotloff, RM .

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 2003, 167 (05) :735-740

[3]

Badesch DB, 2007, J RHEUMATOL, V34, P2417

[4] Pulmonary Arterial Hypertension Baseline Characteristics From the REVEAL Registry [J].

Badesch, David B. ;

Raskob, Gary E. ;

Elliott, C. Greg ;

Krichman, Abby M. ;

Farber, Harrison W. ;

Frost, Adaani E. ;

Barst, Robyn. J. ;

Benza, Raymond L. ;

Liou, Theodore G. ;

Turner, Michelle ;

Giles, Scott ;

Feldkircher, Kathy ;

Miller, Dave P. ;

McGoon, Michael D. .

CHEST, 2010, 137 (02) :376-387

[5] Longterm Survival Among Patients with Scleroderma-associated Pulmonary Arterial Hypertension Treated with Intravenous Epoprostenol [J].

Badesch, David B. ;

McGoon, Michael D. ;

Barst, Robin J. ;

Tapson, Victor F. ;

Rubin, Lewis J. ;

Wigley, Fredrick M. ;

Kral, Kenneth M. ;

Raphiou, Ibrahim H. ;

Crater, Glenn D. .

JOURNAL OF RHEUMATOLOGY, 2009, 36 (10) :2244-2249

[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] Echocardiography in Pulmonary Arterial Hypertension: from Diagnosis to Prognosis [J].

Bossone, Eduardo ;

D'Andrea, Antonello ;

D'Alto, Michele ;

Citro, Rodolfo ;

Argiento, Paola ;

Ferrara, Francesco ;

Cittadini, Antonio ;

Rubenfire, Melvyn ;

Naeije, Robert .

JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY, 2013, 26 (01) :1-14

[8]

Bryan C, 1996, BRIT J RHEUMATOL, V35, P1122

[9] Multicentre randomised placebo-controlled trial of oral anticoagulation with apixaban in systemic sclerosis-related pulmonary arterial hypertension: the SPHInX study protocol [J].

Calderone, Alicia ;

Stevens, Wendy ;

Prior, David ;

Nandurkar, Harshal ;

Gabbay, Eli ;

Proudman, Susanna M. ;

Williams, Trevor ;

Celermajer, David ;

Sahhar, Joanne ;

Wong, Peter K. K. ;

Thakkar, Vivek ;

Dwyer, Nathan ;

Wrobel, Jeremy ;

Chin, Weng ;

Liew, Danny ;

Staples, Margaret ;

Buchbinder, Rachelle ;

Nikpour, Mandana .

BMJ OPEN, 2016, 6 (12)

[10] Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study [J].

Channick, RN ;

Simonneau, G ;

Sitbon, O ;

Robbins, IM ;

Frost, A ;

Tapson, VF ;

Badesch, DB ;

Roux, S ;

Rainisio, M ;

Bodin, F ;

Rubin, LJ .

LANCET, 2001, 358 (9288) :1119-1123

← 1 2 3 4 5 6 7 →