title: Current Approaches to the Treatment of Systemic-Sclerosis-Associated Pulmonary Arterial Hypertension (SSc-PAH).
abstract: Pulmonary arterial hypertension (PAH) is a severe condition causing significant morbidity and mortality in patients with systemic sclerosis (SSc). Despite the use of specific treatments, SSc-PAH survival remains poorer than in idiopathic PAH (IPAH). Recent therapeutic advances in PAH show a lower magnitude of response in SSc-PAH and a higher risk of adverse events, as compared to IPAH. The multifaceted underlying mechanisms and the multisystem nature of SSc probably explain part of the worse outcomes in SSc-PAH compared to IPAH. This review describes the current management of SSc-PAH with an emphasis on the impact of the different organ involvements in the prognosis and treatment response. An earlier detection of PAH and a better characterization of the clinical phenotypes of SSc-PAH are warranted in clinical practice and future trials. Determinants of prognosis, surrogate markers of clinical improvement or worsening, and relevance of the common endpoints used in clinical trials should be evaluated in this specific population. A multidisciplinary approach in expert referral centers is mandatory for SSc-PAH management.
authors: U995 Lille Inflammation Research International Center, University Lille, Lille, France.|Département de Médecine Interne et Immunologie Clinique, CHU Lille, Lille, France.|Centre National de Référence Maladies Systémiques et Auto-Immunes Rares (Sclérodermie Systémique), Lille, France.|FHU Immune-Mediated Inflammatory Diseases and Targeted Therapies (IMMINeNT), Lille, France.
authors: Vincent
authors: V
authors: Sobanski
authors: U995 Lille Inflammation Research International Center, University Lille, Lille, France.|Département de Médecine Interne et Immunologie Clinique, CHU Lille, Lille, France.|Centre National de Référence Maladies Systémiques et Auto-Immunes Rares (Sclérodermie Systémique), Lille, France.|FHU Immune-Mediated Inflammatory Diseases and Targeted Therapies (IMMINeNT), Lille, France.
authors: David
authors: D
authors: Launay
authors: U995 Lille Inflammation Research International Center, University Lille, Lille, France.|Département de Médecine Interne et Immunologie Clinique, CHU Lille, Lille, France.|Centre National de Référence Maladies Systémiques et Auto-Immunes Rares (Sclérodermie Systémique), Lille, France.|FHU Immune-Mediated Inflammatory Diseases and Targeted Therapies (IMMINeNT), Lille, France.
authors: Eric
authors: E
authors: Hachulla
authors: Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin Bicêtre, France. marc.humbert@aphp.fr.|Service de Pneumologie, Hôpital Bicêtre, AP-HP, Le Kremlin Bicêtre, France. marc.humbert@aphp.fr.|INSERM UMR S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France. marc.humbert@aphp.fr.
authors: Marc
authors: M
authors: Humbert
mesh_terms: D002121:Calcium Channel Blockers / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D065128:Endothelin Receptor Antagonists / Q000627:therapeutic use; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology*; D016040:Lung Transplantation; D009569:Nitric Oxide / Q000502:physiology; D012595:Scleroderma, Systemic / Q000150:complications* / Q000188:drug therapy / Q000453:epidemiology
chemical_list: D002121:Calcium Channel Blockers; D065128:Endothelin Receptor Antagonists; D009569:Nitric Oxide
keywords: Connective tissue disease; Pulmonary arterial hypertension; Systemic sclerosis; Treatment