Abstract
BackgroundPrevious 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.MethodsFifty 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.ResultsBaseline 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).ConclusionBaseline 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.
Highlights
Pulmonary arterial hypertension (PAH) is a lifethreatening and refractory disease, associated with progressive pulmonary vascular remodeling and increased pulmonary vascular resistance, leading to right ventricular failure and death
The association between UA and Connective tissue disease (CTD)-PAH has been previously described, and up to date, the evidence suggests that: 1) UA levels above 357 μmol/L are associated with PAH in systemic lupus erythematosus (SLE) [20]; 2) Serum UA levels are correlated with disease severity in PAH secondary to systematic sclerosis (SSc-PAH) [21]; 3) Serum baseline UA levels may predict clinical outcome in patients with Pulmonary hypertension secondary to connective tissue disease (CTD-pulmonary hypertension (PH)) [18]
Baseline characteristics of the normouricemia and the hyperuricemia groups (Table 1) The 50 patients were separated into two groups based on serum UA levels: normouricemia group (UA: women < 357 μmol/L, men < 420 μmol/L) and hyperuricemia group (UA: women ≥357 μmol/L, men ≥420 μmol/L)
Summary
Pulmonary arterial hypertension (PAH) is a lifethreatening and refractory disease, associated with progressive pulmonary vascular remodeling and increased pulmonary vascular resistance, leading to right ventricular failure and death. The association between UA and CTD-PAH has been previously described, and up to date, the evidence suggests that: 1) UA levels above 357 μmol/L are associated with PAH in systemic lupus erythematosus (SLE) [20]; 2) Serum UA levels are correlated with disease severity in PAH secondary to systematic sclerosis (SSc-PAH) [21]; 3) Serum baseline UA levels may predict clinical outcome in patients with CTD-PH [18]. Few studies have explored the value of serum UA in pulmonary arterial hypertension secondary to connective tissue disease (CTDPAH) This retrospective study aimed to investigate the clinical value of serum UA levels in patients with CTD-PAH
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