BACKGROUND/
AIMS: We investigated whether
transthoracic echocardiography -suspected
pulmonary hypertension (
PH )
affects survival in
systemic lupus erythematosus (SLE)
patients and examined factors associated with
PH occurrence and
survival .
METHODS: This retrospective single-center study included 154 Korean SLE
patients fulfilling the American College of
Rheumatology criteria (January 1995 to June 2013).
Student t
test, Mann-Whitney U test, Kaplan-Meier curves, and log-rank tests were used for comparisons.
RESULTS: A total of 35 SLE
patients with
PH (SLE/
PH +) and 119 without
PH (SLE/
PH -) were analyzed. Higher percentages of
interstitial lung disease , Raynaud's phenomenon (RP),
World Health Organization functional
classification III/IV, and
cardiomegaly were found in SLE/
PH + compared to SLE/
PH -. Furthermore, the Systemic Lupus International Collaborating Clinics/American College of
Rheumatology Damage Index was significantly higher in SLE/
PH + (2.46 +/- 1.245 vs. 1.00 +/- 1.235), whereas
survival rates were significantly higher in SLE/
PH - in log-rank tests (p = 0.001). In
multivariate analysis , the adjusted
mortality hazard ratio (HR) for SLE/
PH +
patients was 3.10. Subgroup
analysis demonstrated a higher percentage of
lupus nephritis in the SLE/
PH +
patients who died (p = 0.039) and low
complement -3 levels (p = 0.007). In univariate
analysis , the
mortality HR for SLE/
PH +
patients with
lupus nephritis was 4.62, whereas the presence of RP decreased the
mortality risk in
multivariate analysis ; adjusted HR, 0.10.
CONCLUSIONS: PH is an independent factor predicting
survival in SLE
patients . The presence of
lupus nephritis resulted in an increased trend for
mortality , whereas coexistence of RP was associated with a better
survival prognosis in SLE/
PH +
patients .