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Background  Previous studies have shown that serum uric acid (UA) levels are correlated with the severity of idiopathic pulmonary arterial hypertension (IPAH) and are predictors of disease prognosis. Still, few studies have explored the value of serum UA in pulmonary arterial hypertension secondary to connective tissue disease (CTD-PAH). This retrospective study aimed to investigate the clinical value of serum UA levels in patients with CTD-PAH.    Methods  Fifty CTD-PAH patients were enrolled in our study, from which baseline UA levels, respective variations, and additional clinical data were collected. The potential association between baseline UA level and severity of CTD-PAH was investigated. Furthermore, the relationship between baseline UA and survival rate of CTD-PAH patients, as well as between UA variations and survival rate of pulmonary hypertension secondary to connective tissue disease (CTD-PH) patients was discussed.    Results  Baseline serum UA levels were positively correlated with pulmonary vascular resistance (PVR). During the follow-up period, 3 CTD-PAH and 12 CTD-PH patients died. Kaplan-Meier survival curves showed lower survival rate in patients with hyperuricemia than in patients with normouricemia, in both groups (CTD-PAH group  p  = 0.041, CTD-PH group  p  = 0.013). Concerning serum UA variations, patients with persistent hyperuricemia showed the lowest survival rate when compared with patients with steady normouricemia ( p  = 0.01) or patients with decresing serum UA levels, i.e. undergoing from a status of hyperuricemia to a status of normouricemia ( p  = 0.023).    Conclusion  Baseline serum UA levels might predict severity of CTD-PAH. Together with baseline values, changes of uric acid level may predict the clinical prognosis of the disease.

bmc pulmonary medicine

Serum uric acid is associated with disease severity and may predict clinical outcome in patients of pulmonary arterial hypertension secondary to connective tissue disease in Chinese: a single-center retrospective study