Background/Purpose: Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease characterized by fluctuating disease activity over periods of years. The estimated 5-year survival rate of patients with SLE has improved greatly from 50% to over 90% over the last several decades, and the 10-year survival rate has also increased to nearly 90%. In patients who survive longer than 10 years, the major cause of death has become not simply active SLE itself, and recently the management of SLE has been aimed at not only remission induction and control of disease activity, but also long-term prevention of organ damage resulting from treatment and complications. In SLE patients, organ damage has been principally assessed using the Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index (SDI), which has been extensively validated. In this study, we examined the long-term outcome, causes of death, and factors associated with damage accrual using the SDI in a single-center cohort of SLE patients.

Methods: We examined a cohort of 557 patients who had been referred to Niigata University Hospital and diagnosed as having SLE between 1961 and 2013. The patients’ data at the latest visit were collected from their clinical records on April 2014, and causes of death were also defined on the basis of those data. Survival from the time of diagnosis was calculated by the Kaplan-Meier method. The SDI was calculated, and analyzed using Spearman’s correlation coefficient and stepwise multiple regression analysis to reveal the factors associated with any organ damage.

Results: Data from 458 of the patients were successfully obtained. The overall 5-year survival rate was 89.9%, the 10-year survival rate was 86.0%, the 20-year survival rate was 78.6%, and the 30-year survival rate was 76.4%. The patients diagnosed after 2000 had a significantly high 5-year survival rate of 97.1%. Among 115 patients who died during the observation period, the main causes of death were infections (22.6%), vascular disease (22.6%), and SLE-related complications (21.7%). Of the 343 patients who were alive at the time of this study, common complications were hypertension (40.8%), dyslipidemia (26.8%), diabetes mellitus (15.7%), and osteonecrosis (11.1%). The mean SDI was 0.80±1.07 and was positively correlated with age (r=0.2963, p<0.001), disease duration (r=0.1459, p<0.001), and hypertension (r=0.2299, p<0.001), whereas it was negatively correlated with administration of bisphosphonate (r=-0.115, p=0.049). Stepwise multiple regression analysis selected age (standardized beta=0.2762, p<0.001), hypertension (standardized beta=0.2267, p<0.001), and antiphospholipid antibody syndrome (standardized beta=0.1533, p=0.005) as positive independent variables, whereas administration of bisphosphonate (standardized beta=-0.1295, p=0.016) was selected as a negative independent variable.

Conclusion: These results suggest the significance of disease control as well as management of chronic complications such as hypertension and osteoporosis to prevent organ damage in SLE patients receiving long-term treatment.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/long-term-prognosis-and-factors-associated-with-damage-accrual-in-japanese-patients-with-systemic-lupus-erythematosus/