Patients with pulmonary arterial hypertension with CTD exhibit 59% 10-year survival rate
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Key takeaways:
- Overall 10-year prognosis for patients with PAH-CTD is “poor.”
- Independent risk factors for 10-year survival include systemic sclerosis and baseline 6-minute walking distance.
Patients with pulmonary arterial hypertension associated with connective tissue disease demonstrate a “poor” 10-year rate of 59.9%, according to data published in Rheumatology.
“Pulmonary arterial hypertension associated with connective tissue disease (CTD-PAH)
accounts for approximately 20% to 30% of PAH cases,” Xiaoxi Chen, MD, of the department of pulmonary vascular disease at the National Center for Cardiovascular Disease, part of Fuwai Hospital, in Beijing, China, and co-authors wrote. “Evidence of the 10-year survival rate and corresponding prognostic factors are still scarce in CTD-PAH patients.”
To report 10-year survival rates in patients with CTD-PAH, as well as evaluate prognostic indicators, Chen and colleagues collected data from a national Chinese prospective registry. Patients were included in the analysis if they experienced a right heart catheterization 1 month before, or during, the study enrollment period. Enrollment began in August 2009 and ran through December 2019.
The researchers collected baseline and follow-up information, with patient statuses confirmed via phone as well as outpatient or inpatient hospital visits. Risk stratification was conducted using several noninvasive measures.
The analysis included a total of 266 patients with CTD-PAH, including 94 with systemic lupus erythematosus, 63 with primary Sjögren’s syndrome, 33 with mixed CTD and 21 with systemic sclerosis.
At 10 years, the estimated rate of survival among included patients was 59.9% (95% CI, 51.5-69.7). The 1-year survival rate was estimated at 92.1% (95% CI, 88.8-95.5), while the survival rate for 3 years was 82.7% (95% CI, 78-87.6) and the rate at 5 years was 74.1% (95% CI, 68.5-80.1). Risk factors for 10-year survival were underlying connective tissue disease — specifically systemic sclerosis — as well as baseline 6-minute walking distance and SaO2.
In the years between 2015 and 2019, more patients initiated therapy for CTD and PAH at the time of diagnosis, signaling a stricter adherence to published guidelines for disease management, the researchers added.
“The present study reports the 10-year survival rate of Chinese CTD-PAH patients for the first time,” Chen and colleagues wrote. “The 10-year survival was poor, which indicated the devastating nature of CTD-PAH. There was a tendency for more standardized treatment profiles and better 1- and 3-year survival after 2015.
“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,” they added.
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