[Update in pulmonary hypertension associated with connective tissue diseases - a systematic literature review]

Pulmonary hypertension (PH) is a common complication of connective tissue disease. While there are no reliable epidemiological data, the prevalence of systemic sclerosis (SSc) has been estimated as being 8-12%. Mixed connective tissue disease (MCTD) and systemic lupus erythematodes (SLE) are thought to have a lower prevalence. PH associated with SSc has a poor prognosis, a two-year survival rate of approximately 50%, if untreated. Systematic literature search for studies of PH and CTD between 02/2007 and 02/2008 found 38 articles, a selection of which is reviewed here. One epidemiological study showed that nowadays PH is together with interstitial pulmonary fibrosis the most common cause of death in patients with SSc. Before the introduction of angiotensin- converting enzyme inhibitors the most frequent cause of death was acute renal crisis. Investigations of the pathogenesis of PH in CTD revealed that in patients with a severe, treatment-resistant course there is frequent histological evidence of pulmonary veno-occlusive disease. A prospective study on diagnosis revealed that transthoracic echocardiography (TTE) is better than magnetic resonance imaging or pulmonary function tests especially in screening for PH in patients with SSc, because of its high specificity and good predictive value at higher pulmonary pressures. But because of the low sensitivity of TTE right heart catheterization is the gold standard for verifying PH also in patients with SSc. A therapeutic uncontrolled trial indicated that treatment with bosentan combined with sildenafil results in clinical stabilization, but patients with idiopathic PHT responded better.