lu-208075 and Lung-Diseases--Interstitial

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.

Topics: Antihypertensive Agents; Bosentan; Connective Tissue Diseases; Endothelin Receptor Antagonists; Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial; Lung Transplantation; Phenylpropionates; Phosphodiesterase 5 Inhibitors; Prognosis; Pyridazines; Scleroderma, Systemic; Sulfonamides