الفهرس 
INTRODUCTIONOnly 14 pages are availabe for public view
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Abstract
Systemic lupus erythematosus (SLE) is an autoimmune disease that affects various body organs and can range from mild symptoms to severe disease.
It occurs in 20-150 per 100,000 and primarily affects young females, with a female-to-male ratio of 7:1 to 15:1. Studies on SLE prevalence and mortality rates in Egypt and the Middle East are limited, but observations suggest significant morbidity and high hospital admission rates, particularly from lupus nephritis. The etiology of SLE is not fully understood, but it is believed to be influenced by genetic, environmental, and immunological factors.
Clinical presentation of SLE is highly variable ranging from constitutional, joint, and skin symptoms to serious, life-threatening organ affection.
Lupus nephritis (LN) is a common and devastating manifestation of SLE, affecting 30-50% of lupus patients. Progression to chronic kidney disease (CKD), end-stage kidney disease (ESKD), and mortality is high, particularly in proliferative glomerulonephritis. ESKD is estimated to develop in 11-12% of patients at 5 years of LN diagnosis.
The BAFF pathway is crucial for B cell maturation, survival, and activation in LN. BAFF is a member of the TNF superfamily. They promote B cell survival and differentiation, preventing apoptosis and promoting autoantibody production. They also contribute to the differentiation of B cells into antibody-secreting plasma cells, further promoting autoantibodies production. They also play a role in T cell activation, indirectly stimulating T cells by promoting antigen-presenting cells (APCs). The BAFF pathway can stimulate the production of pro-inflammatory cytokines and exacerbate the inflammatory response in the kidneys.
Therapeutic strategies have been developed to target this pathway, such as belimumab, a monoclonal antibody that inhibits BAFF, which has shown promise in reducing disease activity in lupus nephritis patients.
Lupus nephritis can manifest in various clinical forms, including asymptomatic or minimally symptomatic disease, hypertension and edema, nephrotic syndrome, chronic renal insufficiency, and rapidly progressive GN. Controlling these conditions is crucial to prevent end-organ damage and improve overall health.
Lupus nephritis can be diagnosed and monitored using various tests such as urine analysis, serum creatinine, anemia monitoring, anti-dsDNA antibodies, complement levels, imaging, and kidney biopsy. The gold standard for diagnosing LN is kidney biopsy. Lupus nephritis is classified into classes I-VI based on pathological features with class III and IV being the most proliferative.
The FDA has approved Belimumab and Voclosporin as additional treatments for LN. Belimumab is a monoclonal antibody that inhibits B-lymphocyte stimulator activity, helping to reduce lupus disease activity, flares, steroid use, and disease damage. Studies show that Belimumab significantly achieves urinary protein-to-creatinine ratios of 0.7 or less and an eGFR of 20% or less. It also reduces the risk of kidney-related events and death. Extension studies confirmed its effectiveness.