Characteristics and Long-term Outcomes of Patients with Lupus-related Protein-losing Enteropathy: A Retrospective Study

Objectives: The long-term outcomes of patients with systemic lupus erythematosus (SLE)-related protein-losing enter-opathy (PLE) are unclear. This study was aimed to investigate the clinical characteristics and long-term outcomes of patients with SLE-related PLE.

Methods: This retrospective cohort study enrolled 58 patients with SLE-related PLE who were admitted to our center from January 2000 to June 2016. The patients' baseline characteristics and follow-up data were analyzed, and the prognostic outcomes were survival and disease flares. The prognoses were analyzed using Kaplan-Meier curves, log-rank tests, and Cox regression models. Factors with values of P<0.05 were considered potential predictors.

Results: Two-thirds of patients had intestinal symptoms, and 77.6% of patients had concomitant organ/system involvement, including serositis (77.6%), lupus nephritis (57.6%), neuropsychiatric lupus (10.3%), and hematological disorders (22.4%). Common abnormalities in the laboratory test results were hypocomplementemia (87.9%), anti-Sjögren syndrome antigen A antibodies (51.7%), and high total cholesterol levels (62.1%). Five flares were recorded in 47 patients. The 1-, 3-, and 5-year survival rates were 93.6%, 91.3%, and 88.4%, respectively. Infection was the cause of death in 60% of patients. High 24-hour urine protein level was an independent risk factor associated with death (P = 0.012). Severe hypoalbuminemia (<12 g/L) was a predictor of disease flares in SLE-related PLE (hazard ratio, 10.345; 95% confidence interval, 1.690-63.321).

Conclusions: Infection causes most of the deaths in patients with SLE-related PLE. High 24-hour urine protein level is an independent risk factor associated with death, and severe hypoalbuminemia is a predictor of disease flares.