Pulmonary Hypertension How to Best Treat the Different Scleroderma Phenotypes?

被引:1
作者
Korman, Benjamin D. [1 ]
Lachant, Daniel J. [2 ]
Castelino, Flavia, V [3 ]
机构
[1] Univ Rochester, Div Allergy Immunol & Rheumatol, Med Ctr, 601 Elmwood Ave,Box 695, Rochester, NY 14642 USA
[2] Univ Rochester, Div Pulm & Crit Care Med, Med Ctr, 601 Elmwood Ave,Box 692, Rochester, NY 14642 USA
[3] Massachusetts Gen Hosp, Div Rheumatol, 55 Fruit St,Yawkey 4B, Boston, MA 02114 USA
关键词
Scleroderma; Systemic sclerosis; Pulmonary hypertension; Screening; Treatment; RIGHT-HEART CATHETERIZATION; SYSTEMIC-SCLEROSIS PATIENTS; BASE-LINE CHARACTERISTICS; ARTERIAL-HYPERTENSION; IMMUNOSUPPRESSIVE THERAPY; FERRIC CARBOXYMALTOSE; COMBINATION THERAPY; DISEASE; DIAGNOSIS; AMBRISENTAN;
D O I
10.1016/j.rdc.2023.01.015
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Pulmonary hypertension in SSc is a multifactorial illness, which can have features of any of the WHO PH classes and often results from more than one pathophysiology. Advances in diagnosis and screening have allowed for earlier detection and treatment of less severe disease, which should improve outcomes. Treatment modalities are evolving and most patients will require a combination of therapies and care from an interdisciplinary team of specialists in rheumatology, pulmonology, and cardiology. A better understanding of disease mechanisms has led to novel diagnostic and treatment strategies to specifically address each patient's PH phenotype and improve outcomes in this challenging disease.
引用
收藏
页码:345 / 357
页数:13
相关论文
共 50 条
[31]   Poor survival in patients with scleroderma and pulmonary hypertension due to heart failure with preserved ejection fraction [J].
Bourji, Khalil I. ;
Kelemen, Benjamin W. ;
Mathai, Stephen C. ;
Damico, Rachel L. ;
Kolb, Todd M. ;
Mercurio, Valentina ;
Cozzi, Franco ;
Tedford, Ryan J. ;
Hassoun, Paul M. .
PULMONARY CIRCULATION, 2017, 7 (02) :409-420
[32]   Improved survival in limited scleroderma-related pulmonary artery hypertension [J].
Marini, Carlo ;
Formichi, Bruno ;
Bauleo, Carolina ;
Michelassi, Claudio ;
Pancani, Roberta ;
Prediletto, Renato ;
Miniati, Massimo ;
Catapano, Giosue ;
Monti, Simonetta ;
Mannucci, Francesca ;
Tavoni, Antonio ;
D'Ascanio, Anna ;
Pastormerlo, Luigi Emilio ;
Giannoni, Alberto ;
Giuntini, Carlo .
INTERNAL AND EMERGENCY MEDICINE, 2014, 9 (04) :385-396
[33]   Iron therapy as a novel treatment of scleroderma-related pulmonary hypertension: A case report and literature review [J].
Neumann, Melissa ;
Wong, Karen A. ;
Lazo, Kevin ;
Stover, Diane .
RESPIROLOGY CASE REPORTS, 2022, 10 (02)
[34]   Pulmonary veno-occlusive disease and scleroderma associated pulmonary hypertension [J].
Johnson, Sindhu R. ;
Patsios, Demetris ;
Hwang, David M. ;
Granton, John T. .
JOURNAL OF RHEUMATOLOGY, 2006, 33 (11) :2347-2350
[35]   Clinical and laboratory features of scleroderma patients with pulmonary hypertension [J].
Yamane, K ;
Ihn, H ;
Asano, Y ;
Yazawa, N ;
Kubo, M ;
Kikuchi, K ;
Soma, Y ;
Tamaki, K .
RHEUMATOLOGY, 2000, 39 (11) :1269-1271
[36]   Resistin-Like Molecule-β in Scleroderma-Associated Pulmonary Hypertension [J].
Angelini, Daniel J. ;
Su, Qingning ;
Yamaji-Kegan, Kazuyo ;
Fan, Chunling ;
Teng, Xingwu ;
Hassoun, Paul M. ;
Yang, Stephen C. ;
Champion, Hunter C. ;
Tuder, Rubin M. ;
Johns, Roger A. .
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY, 2009, 41 (05) :553-561
[37]   Pulmonary hypertension with limited cutaneous scleroderma (CREST syndrome) [J].
Berends, JC ;
Dompeling, EC ;
van der Star, JG ;
Hoorntje, JCA .
NETHERLANDS JOURNAL OF MEDICINE, 2000, 57 (06) :229-232
[38]   Survival protection by bodyweight in isolated scleroderma-related pulmonary artery hypertension [J].
Marini, Carlo ;
Formichi, Bruno ;
Bauleo, Carolina ;
Michelassi, Claudio ;
Air, Edoardo ;
Rossi, Giuseppe ;
Giuntini, Carlo .
INTERNAL AND EMERGENCY MEDICINE, 2016, 11 (07) :941-952
[39]   Pulmonary arterial hypertension screening practices in scleroderma patients among Canadian rheumatologists [J].
Quinn, Ryan ;
Koh, Deborah ;
Kelly, Dylan ;
Beattie, Karen A. ;
Larche, Maggie J. .
JOURNAL OF SCLERODERMA AND RELATED DISORDERS, 2020, 5 (03) :237-241
[40]   The frequency of pulmonary hypertension in juvenile scleroderma [J].
Amra Adrovic ;
Kenan Barut ;
Aida Koka ;
Refet Gojak ;
Tuncalp Demir ;
Funda Oztunc ;
Ozgur Kasapcopur .
Pediatric Rheumatology, 12 (Suppl 1)