Among patients with pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD-PAH), 10-year survival rates are poor, according to findings from a study derived from a Chinese national multicenter, prospective registry and published in Rheumatology.

The researchers sought to accomplish the following:

  • Report the 10-year survival rate and prognostic factors among Chinese patients with CTD-PAH;
  • Show changes in treatment and their effects on survival outcomes between patients enrolled from 2009 to 2014 and those enrolled from 2015 to 2019; and
  • Validate the discriminatory value of the 4-strata risk approach in predicting 10-year survival rates among patients with CTD-PAH.

Individuals who had undergone right heart catheterization within 1 month prior to or during the study enrollment period from August 2009 through December 2019 and who fulfilled the criterion of “mean pulmonary arterial pressure ≥25 mm Hg and pulmonary arterial wedge pressure ≤15 mm Hg” were enrolled consecutively in the study.

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Two hundred sixty-six patients with CTD-PAH were enrolled in the current study. Participants were from 23 tertiary clinical centers located in 13 Chinese provinces. The median patient age was 36.0 years; 97% of the participants were female. In the CTD-PAH study cohort, the 5 major CTD subgroups were as follows:

  • Systemic lupus erythematosus: 35.3%
  • Primary Sjögren syndrome: 23.7%
  • Mixed CTD: 12.4%
  • Systemic sclerosis: 7.9%
  • Rheumatoid arthritis: 6.4%

Regarding PAH-targeted baseline therapy, 17.3% of participants received combination therapy and 57.5% received monotherapy; 25.2% of the patients did not receive any targeted agents at baseline. Further, glucocorticoids and immunosuppressants were used by 31.2% and 23.7% of participants, respectively, with 19.5% of them receiving intensive immunosuppressive therapy. In addition, 23.7% of the participants received binary therapy (ie, both PAH-targeted therapy and CTD therapy simultaneously).

Over a maximum follow-up of 11.58 years (median, 4.85 years), 72 of the participants died and 10 of 266 were lost to follow-up. The main cause of death was right heart failure. At 10 years, the survival estimate was 59.9% (95% CI, 51.5-69.7), compared with 1-, 3-, and 5-year survival estimates of 92.1% (95% CI, 88.8-95.5), 82.7% (95% CI, 78.0-87.6), and 74.1% (95% CI, 68.5-80.1), respectively.

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Patients with systemic sclerosis-PAH had the poorest estimated 10-year survival rate of 26.8%, whereas in those with systemic lupus erythematosus-PAH, the 10-year survival rate was 64.3%.

Of note, 1-year survival increased from 89.8% over 2009 to 2014 to 93.9% over 2015 to 2019. Three-year survival rose from 80.1% in 2009 to 2014 to 86.5% in 2015 to 2019. The 4-strata approach performed well in predicting 10-year survival at follow-up (C-index=0.742).

“Continued efforts should be made to guarantee regular comprehensive evaluation and a sufficient course of treatment in both CTD and PAH management in the whole CTD-PAH course,” the authors concluded.


Chen X, Quan R, Qian Y, et al. 10-year survival of pulmonary arterial hypertension associated with connective tissue disease: insights from a multicenter PAH registry. Rheumatology. Published online March 13, 2023. doi:10.1093/rheumatology/kead103