title: Pulmonary hypertension complicating connective tissue disease.
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.
authors.affiliation: Division of Pulmonary, Critical Care Medicine, Clinical Immunology, and Allergy, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California.
authors.forename: Joseph P
authors.initials: JP
authors.lastname: Lynch
authors.forename: John A
authors.initials: JA
authors.lastname: Belperio
authors.forename: Rajeev
authors.initials: R
authors.lastname: Saggar
authors.forename: Michael C
authors.initials: MC
authors.lastname: Fishbein
authors.forename: Rajan
authors.initials: R
authors.lastname: Saggar
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use; D000077300:Bosentan; D003240:Connective Tissue Diseases / Q000150:complications / Q000503:physiopathology; D065128:Endothelin Receptor Antagonists; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D017563:Lung Diseases, Interstitial / Q000150:complications; D016040:Lung Transplantation; D010666:Phenylpropionates / Q000627:therapeutic use; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use; D011379:Prognosis; D011724:Pyridazines / Q000627:therapeutic use; D012595:Scleroderma, Systemic / Q000150:complications* / Q000401:mortality / Q000503:physiopathology; D013449:Sulfonamides / Q000627:therapeutic use
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D010666:Phenylpropionates; D058986:Phosphodiesterase 5 Inhibitors; D011724:Pyridazines; D013449:Sulfonamides; C467894:ambrisentan; D000077300:Bosentan