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Background  To study the oxygen uptake efficiency and determine usefulness of submaximal parameters of oxygen uptake in systemic lupus erythematosus associated pulmonary arterial hypertension (SLE PAH) on performing a cardiopulmonary exercise test (CPET).    Methods  CPET was performed in 21 SLE PAH patients, equal number of idiopathic pulmonary arterial hypertension (IPAH) patients and controls. Peak VO 2 , anaerobic threshold (AT), oxygen uptake efficiency slope (OUES) and oxygen uptake efficiency plateau (OUEP) and other CPET parameters were examined. All subjects had pulmonary function test (PFT) at rest, which included FEV 1 , FVC, FEV 1 /FVC, DLCO measurements. Right heart catheterization (RHC) was also done in SLE PAH and IPAH patients. CPET parameters were compared with RHC parameters to determine potential correlations.    Results  Peak VO 2 , PETCO 2  and peak O 2  pulse were lower in SLE PAH than IPAH and controls with OUE being lower during all stages of exercise in SLE PAH. DLCO and FVC values were significantly lower in SLE PAH ( p  < 0.05). Peak O 2  pulse and VO 2 @AT in SLE PAH and IPAH was low ( p  < 0.05) and significant difference between SLE PAH and IPAH was seen ( p  < 0.05). PVR correlated with the lowest V E /VCO 2 , O 2  pulse, peak PETCO 2  and OUE in SLE PAH patients (all  p  < 0.05).    Conclusions  SLE PAH patients have cardiopulmonary exercise limitation with reduced oxygen uptake efficiency. VO 2 @ at AT, peak O 2  pulse and O 2  pulse at AT were significantly reduced ( p  < 0.05). Key CPET parameters correlated with elevated pulmonary vascular resistance (PVR). Submaximal parameters of oxygen uptake are equally useful in SLE PAH.

Characteristics of exercise capacity in female systemic lupus erythematosus associated pulmonary arterial hypertension patients