Abstract

Objective: To investigate the association between anti-phospholipid syndrome (APS) and the risk of newly diagnosed systemic lupus erythematosus (SLE).Methods: We used 2003–2013 data derived from Taiwan's National Health Insurance Research Database to conduct this nationwide, population-based. We identified AS patients newly diagnosed between 2005 to 2013 as the study group and applied age-sex matched (1:20) and propensity score-matched (PSM) (1:2) non-SLE individuals as controls. The association between APS and risk of incident SLE was determined by calculating hazard ratios (HRs) with 95% confidence intervals (CIs) using Cox proportional hazard regression analysis.Results: We identified 1,245 patients with APS as well as 24,900 age- and sex-matched non-APS controls and 727 APS patients as well as 1,454 PSM non-APS controls. We found that the risk for incident SLE in the APS group was 80.70 times higher than the non-APS group, and the association remained robust after PSM (HR, 28.55; 95% CI, 11.49–70.91). The increased risk for SLE in patients with APS mainly existed within 5 years after the diagnosis of APS. The sensitivity analyses found that the risk for SLE in patients with APS was consistent excluding patients with ITP/AIHA and using distinct definitions of SLE.Conclusion: The present population-based study revealed a robust association between SLE risk and recent APS and highlights the need for vigilance of SLE-associated symptoms in patients who had been diagnosed with APS.

Highlights

  • Systemic lupus erythematosus (SLE) can manifest with haematological and vascular abnormalities prior to the diagnosis of SLE, and recent studies including our study have found an increased risk for SLE in patients with haematological abnormalities, including immune thrombocytopenia (ITP) and autoimmune hemolytic anaemia (AIHA) [1, 2]

  • In the propensity-matched subjects, we enrolled 727 Anti-phospholipid syndrome (APS) patients and 1,454 propensity-score matching (PSM)-matched non-APS controls to address the risk for SLE in patients with APS (Figure 1)

  • After adjustment of the potential confounders, including comorbidities, urbanisation level, history of thromboembolism, pregnancy morbidities, autoimmune hemolytic anaemia, and immune thrombocytopenia, we found that APS was independently associated with incident SLE (HR 80.70; 95% CI 51.37–126.77) (Table 2, Supplementary Table 1)

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Summary

Introduction

Systemic lupus erythematosus (SLE) can manifest with haematological and vascular abnormalities prior to the diagnosis of SLE, and recent studies including our study have found an increased risk for SLE in patients with haematological abnormalities, including immune thrombocytopenia (ITP) and autoimmune hemolytic anaemia (AIHA) [1, 2]. APS and SLE are two closely correlated autoimmune diseases with overlapped clinical and biological characteristics [3,4,5]. Cervera et al conducting a 10-year-follow-up study among 1,000 patients with APS in 13 European countries, reported that 36.2% of APS was associated with SLE [7]. The anti-phospholipid antibodies were found in ∼40% of patients with SLE [6, 8]. SLE-associated APS was found to be more likely to have thrombocytopenia than patients with primary APS [7, 9].

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