Preventing the development of SLE: identifying risk factors and proposing pathways for clinical care

Abstract

Although challenging, developing evidence-based approaches to an early and accurate diagnosis of systemic lupus erythematosus is a key approach to preventing disease and lupus-associated morbidity and mortality. Advances in our understanding of preclinical and incomplete lupus erythematosus have enabled the identification of risk factors that may predict disease and the development of potential strategies aimed at primary prevention. Emerging data support the notion that there is a temporal disease progression from initial asymptomatic autoimmunity (preclinical lupus) through early clinical features of the disease (incomplete lupus erythematosus) to finally becoming fully classifiable systemic lupus erythematosus (complete lupus erythematosus). Here, we review the demographic, clinical, biomarker as well as genetic and environmental features that are reported to increase the risk of disease progression. Based on these risk factors, we propose a clinical care pathway for patients with early disease. We envisage that such a pathway, through early identification of disease, may improve patient outcomes, while reducing health care costs.

Clinical and Immunologic Profiles in Incomplete Lupus Erythematosus and Improvement with Hydroxychloroquine Treatment

“…Use of HCQ in early disease stages has been proposed as a preventive strategy in SLE [15]. To develop insights into this approach, the SLE and ILE patients were further examined in subgroups defined by current HCQ usage.…”

Antinuclear Antibody–Negative Systemic Lupus Erythematosus in an International Inception Cohort

“…Biomarkers such as autoantibodies and a variety of immune‐related and inflammation‐related molecules can appear years prior to clinical symptoms and/or the diagnosis of SLE and can accrue over time . Therefore, longitudinal studies are needed to evaluate the serologic status of anticellular antibody–negative and isolated CMP–positive patients over time and to evaluate whether the status varies with disease activity, damage accrual, therapeutic interventions, and/or specific substrate assays.…”

Pathogenesis of Human Systemic Lupus Erythematosus: A Cellular Perspective

“…Recent work has shown that serum biomarkers such as type II IFN cytokines appear years before SLE diagnosis, followed by anti-dsDNA autoantibodies and then by type I IFN activity immediately preceding clinical diagnosis, bringing forth the notion that they might be exploited in the potential prevention of SLE [8–10]. These studies support the concept of a gradual chronological disease progression, from a preclinical asymptomatic phase, to incomplete lupus erythematosus, and finally, to complete lupus erythematosus [149]. Clinical and serologic parameters distinguish patients with incomplete lupus from SLE patients, and identification of these parameters is crucial for early management of disease [150–152].…”