Pulmonary hypertension in Takayasu arteritis

To determine the frequency and define the causes of pulmonary hypertension (PH) in patients with Takayasu arteritis (TA).Sixty-four TA patients were evaluated by transthoracic echocardiography (TTE). Having an estimated systolic pulmonary arterial pressure (sPAP) ≥40 mm Hg by echocardiography or if performed, mean PAP ≥25 mm Hg in right heart catheterization was defined as PH. Clinical, imaging and laboratory results of the TA patients were obtained from hospital files.In total, seven (10.9%) patients had PH. Four patients had PH due to left-sided heart disease (group 2 PH), three patients due to pulmonary arterial involvement (PAI; group 4 PH) and one patient due to atrial septal defect (group 1 PH). In one patient, combination of PAI, aortic insufficiency and pulmonary venous return anomaly was present and he was considered to have both group 2 and group 4 PH. PAI was more frequent (42.9% vs 15.7%) in patients with PH but the difference was not statistically significant. The percentage of patients treated with cyclophosphamide and/or biologics was higher in the group with PH as compared to the group without PH (P = 0.015). One patient with group 4 PH had been on pulmonary arterial hypertension (PAH)-specific agents for 8 years.Pulmonary hypertension is not infrequent in TA patients and all the potential causes of PH should be carefully evaluated. Patients with severe or treatment-resistant disease are prone to have PH. PAH-specific agents may be effective in patients with group 4 PH.