Pulmonary Hypertension Complicating Connective Tissue Disease

Elizabeth R. Volkmann; Augustine Chung; Rajan Saggar; John A. Belperio; Joseph P. Lynch III

doi:10.1055/s-0037-1606203

Seminars in Respiratory and Critical Care Medicine, Table of Contents

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

Authors

Elizabeth R. Volkmann^*

¹Division of Rheumatology, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California
Augustine Chung^*

²Division of Pulmonary, Critical Care Medicine, Clinical Immunology, and Allergy, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California
Rajan Saggar

²Division 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

²Division 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

²Division of Pulmonary, Critical Care Medicine, Clinical Immunology, and Allergy, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California

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

Full Text

References

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