5. CONCLUSION
Although evidence from our results requires careful application to
clinical decision making owing to several limitations inherent to
spontaneous reporting databases, our approach using a spontaneous
adverse event reporting database for survival analysis may provide
useful information on treatment efficacies in limited situations such as
the treatment of rare diseases. Among drugs targeting the prostacyclin
pathway, epoprostenol was most associated with long-term survival in the
PAH patients reported in the JADER database. ERAs, especially
macitentan, were associated with improved prognosis compared with
non-ERA users. In contrast, the effect of sildenafil on improvement in
PAH mortality was not observed in our data, suggesting the relative
superiority of tadalafil over sildenafil. Additionally, the long-term
efficacy of anticoagulant therapy with warfarin was confirmed in only
non-epoprostenol users.