Pulmonary arterial hypertension related to connective tissue disease: a review

摘要: PAH associated with connective tissue diseases is significant functional impairment and morbidity, carries it a poor prognosis. The mortality as high 10% to 15% in the first year after diagnosis; making devastating disease. availability of ever-increasing numbers treatment options recent era have improved survival this patient population made early accurate diagnosis more important goal. According Registry Evaluate Early Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL), 1-year, 3-year, 5-year, 7-year rates from time diagnostic right-sided heart catheterization patients were found be 85%, 68%, 57%, 49%, respectively, which considerable improvement since National Institutes Health registry 2 decades previously. In study by Condliffe colleagues, SSC-associated 78%at 1 47% at 3 years. Patients SLE-related much higher rate up 75% Proper screening, diagnosis, can impact reducing morbidity mortality. A small assess outcomes asymptomatic CTD exercise induced suggest that bosentan may safe effective improving hemodynamics these patients. This included only 10 patients, additional randomized trials larger subjects are needed affirm hypothesis. Studies under way find therapeutic modalities form PDGF receptor blockers, VEGF tyrosine kinase inhibitors, endothelial dysfunction multikinase inhibitor Raf-1, serotonin antagonists,and rho inhibitors. Despite these, clinical suspicion,