Pulmonary hypertension in Takayasu arteritis

Sari A., Sener Y. Z., Firat E., Armagan B., Erden A., Oksul M., ...More

International Journal of Rheumatic Diseases, vol.21, no.8, pp.1634-1639, 2018 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Article
  • Volume: 21 Issue: 8
  • Publication Date: 2018
  • Doi Number: 10.1111/1756-185x.13354
  • Journal Name: International Journal of Rheumatic Diseases
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.1634-1639
  • Keywords: large vessel vasculitis, pulmonary hypertension, Takayasu arteritis
  • Gazi University Affiliated: No


© 2018 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, LtdAim: To determine the frequency and define the causes of pulmonary hypertension (PH) in patients with Takayasu arteritis (TA). Method: 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. Result: 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. Conclusion: 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.