Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect many organs, including the skin, joints, the central nervous system and the kidneys. Women of childbearing age and certain racial groups are typically predisposed to developing the condition. Rare, inherited, single-gene complement deficiencies are strongly associated with SLE, but the disease is inherited in a polygenic manner in most patients. Genetic interactions with environmental factors, particularly UV light exposure, Epstein-Barr virus infection and hormonal factors, might initiate the disease, resulting in immune dysregulation at the level of cytokines, T cells, B cells and macrophages. Diagnosis is primarily clinical and remains challenging because of the heterogeneity of SLE. Classification criteria have aided clinical trials, but, despite this, only one drug (that is, belimumab) has been approved for use in SLE in the past 60 years. The 10-year mortality has improved and toxic adverse effects of older medications such as cyclophosphamide and glucocorticoids have been partially offset by newer drugs such as mycophenolate mofetil and glucocorticoid-sparing regimes. However, further improvements have been hampered by the adverse effects of renal and neuropsychiatric involvement and late diagnosis. Adding to this burden is the increased risk of premature cardiovascular disease in SLE together with the risk of infection made worse by immunosuppressive therapy. Challenges remain with treatment-resistant disease and symptoms such as fatigue. Newer therapies may bring hope of better outcomes, and the refinement to stem cell and genetic techniques might offer a cure in the future.

Systemic lupus erythematosus.

There have been significant advances in our understanding of human autoimmunity that have led to improvements in classification and diagnosis and, most importantly, research advances in new therapies. The importance of autoimmunity and the mechanisms that lead to clinical disease were first recognized about 50 years ago following the pioneering studies of Macfarlane Burnett and his Nobel Prize-winning hypothesis of the ‘forbidden clone’. Such pioneering efforts led to a better understanding not only of autoimmunity, but also of lymphoid cell development, thymic education, apoptosis and deletion of autoreactive cells. Contemporary theories suggest that the development of an autoimmune disease requires a genetic predisposition and environmental factors that trigger the immune pathways that lead, ultimately, to tissue destruction. Despite extensive research, there are no genetic tools that can be used clinically to predict the risk of autoimmune disease. Indeed, the concordance of autoimmune disease in identical twins is 12–67%, highlighting not only a role for environmental factors, but also the potential importance of stochastic or epigenetic phenomena. On the other hand, the identification of cytokines and chemokines, and their cognate receptors, has led to novel therapies that block pathological inflammatory responses within the target organ and have greatly improved the therapeutic effect in patients with autoimmune disease, particularly rheumatoid arthritis. Further advances involving the use of multiplex platforms for diagnosis and identification of new therapeutic agents should lead to major breakthroughs within the next decade.

https://onlinelibrary.wiley.com/doi/pdf/10.1111/joim.12395

Human autoimmune diseases: a comprehensive update.

SLE is a chronic inflammatory disease that affects the kidneys in about 50% of patients. Lupus nephritis is a major risk factor for overall morbidity and mortality in SLE, and despite potent anti-inflammatory and immunosuppressive therapies still ends in CKD or ESRD for too many patients. This review highlights recent updates in our understanding of disease epidemiology, genetics, pathogenesis, and treatment in an effort to establish a framework for lupus nephritis management that is patient-specific and oriented toward maintaining long-term kidney function in patients with lupus.

/pdf/update-on-lupus-nephritis-400k9b4g2u.pdf

Update on Lupus Nephritis

Researchers have suggested that health disparities in African American women, including adverse birth outcomes, lupus, obesity, and untreated depression, can be explained by stress and coping. The Strong Black Woman/Superwoman role has been highlighted as a phenomenon influencing African American women's experiences and reports of stress. The purpose of this study was to develop a preliminary conceptual framework for Superwoman Schema (SWS) by exploring women's descriptions of the Superwoman role; perceptions of contextual factors, benefits, and liabilities; and beliefs regarding how it influences health. Analysis of eight focus group discussions with demographically diverse African American women yielded themes characterizing the Superwoman role and personal or sociohistorical contextual factors. Participants reported that the Superwoman role had benefits (preservation of self and family or community) and liabilities (relationship strain, stress-related health behaviors, and stress embodiment). The SWS framework might be used to enhance future research on stress and African American women's health.

Superwoman Schema: African American Women’s Views on Stress, Strength, and Health

Systemic lupus erythematosus: Diagnosis and clinical management.

Understanding the Epidemiology and Progression of Systemic Lupus Erythematosus

Objective

To summarize research pertaining to work disability in lupus patients, discuss challenges patients face applying for federal disability assistance in the US, and make recommendations for clinical and health policy research.



Methods

We searched Medline for articles on work or disability in lupus patients and gathered information from the Social Security Administration and the National Organization of Social Security Claimants' Representatives.



Results

We found 12 publications with employment-related data; 6 included analysis of predictors of work status. The prevalence of inability to work or cessation of work was 15–51% in these studies (3–15 years after diagnosis); 20–32% of patients received disability benefits. Lower education level, higher disease activity, higher disease damage, older age, and higher physical job strain were independent predictors of work disability or work cessation in at least 2 studies. Lupus patients may be less successful than patients with other diseases when applying for federal disability assistance, possibly because medical records may not accurately reflect functional limitations. In addition, symptoms contributing to work disability (e.g., fatigue, pain, neurocognitive dysfunction) may be difficult to assess and quantify.



Conclusion

Work disability in lupus patients is common. Additional research on risk factors for work disability in lupus patients and on strategies for reducing the impact of these factors on work-related activities is needed. The development of better measures and rating scales for the symptoms responsible for work disability in lupus patients and studies of factors influencing the success of obtaining federal disability benefits would also be useful.

Lacie Scofield

Papers

Understanding the Epidemiology and Progression of Systemic Lupus Erythematosus

Employment and disability issues in systemic lupus erythematosus: a review.