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Semin Respir Crit Care Med 2017; 38(05): 619-635
DOI: 10.1055/s-0037-1606203
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Pulmonary Hypertension Complicating Connective Tissue Disease

Elizabeth R. Volkmann   *
1   Division of Rheumatology, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California
,
Augustine Chung   *
2   Division of Pulmonary, Critical Care Medicine, Clinical Immunology, and Allergy, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California
,
Rajan Saggar
2   Division of Pulmonary, Critical Care Medicine, Clinical Immunology, and Allergy, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California
,
John A. Belperio
2   Division of Pulmonary, Critical Care Medicine, Clinical Immunology, and Allergy, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California
,
Joseph P. Lynch III
2   Division of Pulmonary, Critical Care Medicine, Clinical Immunology, and Allergy, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California
› Author Affiliations
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Publication History

Publication Date:
15 October 2017 (online)

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Abstract

Pulmonary hypertension (PH) may complicate connective tissue disease (CTD; particularly systemic sclerosis [scleroderma]), and is associated with increased mortality. More than 70% of cases of PH complicating CTD occur in patients with systemic sclerosis (SSc), which is the major focus of this article. Pulmonary complications (i.e., interstitial lung disease [ILD] and PH) are the leading causes of SSc-related deaths. “Isolated” PH (i.e., without ILD) complicates SSc in 7.5 to 20% of cases; secondary PH may also occur in patients with SSc-associated ILD. Several clinical markers and specific autoantibody profiles have been associated with PH in SSc. The role of PH-specific therapy in improving CTD-PH outcomes is under investigation, as prognosis and responsiveness to therapy appear to be worse in SSc-associated PH compared with idiopathic pulmonary arterial hypertension. We discuss medical therapies for CTD-associated PH and the role of lung transplantation for patients who fail medical therapy.

Keywords

systemic sclerosis - scleroderma - pulmonary hypertension - pulmonary arterial hypertension - connective tissue disease

* Both these authors contributed equally to this work.