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Pulmonary Hypertension in Antiphospholipid Syndrome

  • Antiphospholipid Syndrome (D Erkan, Section Editor)
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Abstract

Pulmonary hypertension (PH) is a rare but life-threatening condition in antiphospholipid syndrome (APS) patients with or without systemic lupus erythematosus (SLE). The definition of PH is based on hemodynamic parameters estimated by transthoracic echocardiography (TTE) and confirmed by right heart catheterization (RHC). New evidence suggests that antiphospholipid antibodies (aPL) in SLE patients increase the risk of PH; however, studies yield conflicting results. Hypotheses regarding the impact of aPL on PH include large vessel and microvascular thrombosis, and endothelial remodeling. Natural history of PH is progressive worsening mainly due to recurrent pulmonary embolism. The management in APS patients includes anticoagulation; patients undergoing pulmonary endarterectomy need to be closely monitored because of an increased risk of thrombotic complications.

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Acknowledgments

The authors would like to acknowledge Dr. Heymonet, Dr. Huttin, Dr. Mandry, Dr. Mohamed, Dr. Selton-Suty, Pr. Chabot, Pr. Chaouat, and Pr. Kaminsky for their involvement in multidisciplinary APS-related PH patient management.

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Conflict of Interest

Stéphane Zuily and Denis Wahl declare that they have no conflicts of interest.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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Authors and Affiliations

  1. Vascular Medicine Division and Regional Competence Centre For Rare Vascular and Systemic Autoimmune Diseases, Centre Hospitalier Universitaire (CHU) Nancy, 54000, Nancy, France

    Stéphane Zuily & Denis Wahl

  2. Institut National de la Santé et de la Recherche Médicale (INSERM), U1116, 54000, Nancy, France

    Stéphane Zuily & Denis Wahl

  3. Université de Lorraine, 54000, Nancy, France

    Stéphane Zuily & Denis Wahl

  4. Centre de Compétences Régional des Maladies Vasculaires Rares et Auto-Immunes, Institut Lorrain du Cœur et des Vaisseaux Louis Mathieu, CHU Nancy, Rue du Morvan, 54511, Vandœuvre-lès-Nancy Cedex, France

    Stéphane Zuily & Denis Wahl

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  1. Stéphane Zuily
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  2. Denis Wahl
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Correspondence to Stéphane Zuily.

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This article is part of the Topical Collection on Antiphospholipid Syndrome

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Zuily, S., Wahl, D. Pulmonary Hypertension in Antiphospholipid Syndrome. Curr Rheumatol Rep 17, 4 (2015). https://doi.org/10.1007/s11926-014-0478-8

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