Lungen- und Atemwegsmedizin

Long−Term Prognosis of Connective Tissue Disease Associated Pulmonary Arterial Hypertension

Abstract

Kazuhiko Nakayama

Objective: Pulmonary Arterial Hypertension (PAH) is a progressive disease characterized by increased pulmonary arterial pressure and pulmonary vascular resistance that can lead to right heart failure. Connective Tissue Disease-Associated PAH (CTD-PAH) often has a worse outcome than idiopathic or Hereditary PAH (I/HPAH), suggesting the presence of non-PAH factors that may affect the prognosis of CTD-PAH patients. This study aimed to identify prognostic factors for CTD-PAH.

Methods: Medical records from the April 1999 through November 2014 period were reviewed to determine the time from treatment initiation to occurrence of a clinically worsening event (hospitalization for PAH exacerbation, progression to WHO Functional Class III or worse, decrease in 6-min walking distance by ≥ 15% from baseline, initiation of parenteral prostanoid therapy, or death), and the time elapsed until death. Data at baseline and final assessment were used to identify prognostic factors associated with these events.

Results: In 36 CTD-PAH patients analyzed, the proportions with no clinically worsening events at 1, 2 and 3 years after treatment initiation were 62, 52 and 45%, with survival rates of 88, 77 and 77%, respectively. In multivariate analysis for survival rate, no variable was identifiable as a prognostic factor. However, baseline hemoglobin, QR pattern in electrocardiogram lead V1, 60-min erythrocyte sedimentation rate and mean pulmonary artery pressure at the final assessment were identified as factors significantly associated with clinical worsening.

Conclusion: Not only hemodynamic status, but also non-PAH factors such as anemia, nutritional status and inflammatory activity of the underlying CTD were suggested to influence the prognosis of CTD-PAH patients. Development of a more multifactorial treatment strategy is necessary.