Pulmonary arterial hypertension with connective tissue diseases

Pulmonary arterial hypertension (PAH) occurs in about 5% of connective tissue disease (CTD) patients. Performing echocardiography, the incidence is much higher at 9.5%, thus confirming the presence of asymptomatic PAH. In 32% of MCTD with PAH, PAH was diagnosed within one year of MCTD diagnosis. Therefore, it is necessary to screen for PAH by echocardiography after the diagnosis of CTD, irrespective of the existence of PAH-related signs and symptoms. The treatments of PAH with CTD are different from those of idiopathic PAH in that the immunosuppressive therapy is expected to alleviate PAH with CTD.