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Long-Term Outcomes in Patients With Connective Tissue Disease-Associated Pulmonary Arterial Hypertension in the Modern Treatment Era: Meta-Analyses of Randomized, Controlled Trials and Observational Registries.

Zsuzsanna H McMahanCarol ZhaoRajan SaggarStephen C MathaiLorinda ChungJ Gerry CoghlanMehul ShahJohn HartneyVallerie McLaughlin
Published in: Arthritis & rheumatology (Hoboken, N.J.) (2021)
Modern therapy provides a similar reduction in morbidity/mortality risk in patients with CTD-PAH when compared to the PAH population overall. Risk of death is higher in CTD-PAH patients than in those with PAH overall, but survival has improved in the last 10 years, which may be related to increased screening and/or new treatment approaches. Early detection of PAH in patients with CTD and up-front intensive treatment are warranted.
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