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Semin Respir Crit Care Med 2013; 34(05): 581-599
DOI: 10.1055/s-0033-1356547
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Pulmonary Hypertension Complicating Connective Tissue Disease

Joseph P. Lynch III
1   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
1   Division of Pulmonary, Critical Care Medicine, Clinical Immunology, and Allergy, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California
,
Rajeev Saggar
2   Division of Pulmonary, Critical Care Medicine, Department of Medicine, St. Joseph Medical Center, Phoenix, Arizona
,
Michael C. Fishbein
3   Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California
,
Rajan Saggar
1   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
Further Information

Publication History

Publication Date:
13 September 2013 (online)

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Abstract

Pulmonary hypertension (PH) may complicate connective tissue disease (CTD), particularly systemic sclerosis (SSc, scleroderma), and markedly increases mortality. More than 70% of cases of PH complicating CTD occur in SSc, which is the major focus of this article. Pulmonary complications (i.e., interstitial lung disease [ILD] and PH) are the leading causes of scleroderma-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 is controversial, as prognosis and responsiveness to therapy are 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 failing medical therapy.

Keywords

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

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