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
  • [21] Pulmonary hypertension in scleroderma and its relation to disease activity
    Rawhya R. Elshereef
    Amal A. Hassan
    Aymen F. Darwish
    Hany T. Asklany
    Lamiaa Hamdy
    Egyptian Rheumatology and Rehabilitation, 2013, 40 (4) : 173 - 180
  • [22] Pulmonary hypertension in scleroderma
    Fonollosa-Pla, Vicent
    Pilar Simeon-Aznar, Carmen
    MEDICINA CLINICA, 2016, 146 (01): : 21 - 23
  • [23] Scleroderma pulmonary arterial hypertension: the same as idiopathic pulmonary arterial hypertension?
    Khan, Sarah L.
    Mathai, Stephen C.
    CURRENT OPINION IN PULMONARY MEDICINE, 2023, 29 (05) : 380 - 390
  • [24] Right ventricular remodeling in idiopathic and scleroderma-associated pulmonary arterial hypertension: two distinct phenotypes
    Kelemen, Benjamin W.
    Mathai, Stephen C.
    Tedford, Ryan J.
    Damico, Rachel L.
    Corona-Villalobos, Cecilia
    Kolb, Todd M.
    Chaisson, Neal F.
    Harris, Traci Housten
    Zimmerman, Stefan L.
    Kamel, Ihab R.
    Kass, David A.
    Hassoun, Paul M.
    PULMONARY CIRCULATION, 2015, 5 (02) : 327 - 334
  • [25] Prevalence of auto-antibodies associated to pulmonary arterial hypertension Cheek for in scleroderma - A review
    Nunes, Jose Pedro L.
    Cunha, Andre C.
    Meirinhos, Tiago
    Nunes, Alzira
    Araujo, Paulo M.
    Godinho, Ana R.
    Vilela, Eduardo M.
    Vaz, Carlos
    AUTOIMMUNITY REVIEWS, 2018, 17 (12) : 1186 - 1201
  • [26] Exercise-Induced Pulmonary Hypertension in Scleroderma Patients: A Common Finding but with Elusive Pathophysiology
    Baptista, Rui
    Serra, Sara
    Martins, Rui
    Salvador, Maria Joao
    Castro, Graca
    Gomes, Manuel
    Santos, Lelita
    Monteiro, Pedro
    Pereira da Silva, Jose A.
    Pego, Mariano
    ECHOCARDIOGRAPHY-A JOURNAL OF CARDIOVASCULAR ULTRASOUND AND ALLIED TECHNIQUES, 2013, 30 (04): : 378 - 384
  • [27] Distinctive metabolomic fingerprint in scleroderma patients with pulmonary arterial hypertension
    Deidda, Martino
    Piras, Cristina
    Dessalvi, Christian Cadeddu
    Locci, Emanuela
    Barberini, Luigi
    Orofino, Susanne
    Musu, Mario
    Mura, Mario Nicola
    Manconi, Paolo Emilio
    Finco, Gabriele
    Atzori, Luigi
    Mercuro, Giuseppe
    INTERNATIONAL JOURNAL OF CARDIOLOGY, 2017, 241 : 401 - 406
  • [28] Telangiectases in Scleroderma: A Potential Clinical Marker of Pulmonary Arterial Hypertension
    Shah, Ami A.
    Wigley, Fredrick M.
    Hummers, Laura K.
    JOURNAL OF RHEUMATOLOGY, 2010, 37 (01) : 98 - 104
  • [29] Pulmonary hypertension screening in systemic sclerosis: The best strategy remains to be defined
    Launay, D.
    Sobanski, V.
    REVUE DE MEDECINE INTERNE, 2017, 38 (08): : 499 - 501
  • [30] Limitations to the 6-Minute Walk Test in Interstitial Lung Disease and Pulmonary Hypertension in Scleroderma
    Garin, Margaret C.
    Highland, Kristin B.
    Silver, Richard M.
    Strange, Charlie
    JOURNAL OF RHEUMATOLOGY, 2009, 36 (02) : 330 - 336