Refractory Proliferative Lupus Nephritis

Abstract

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by abnormal immunologic function and the production of autoantibodies against self-antigens resulting in chronic inflammation that can manifest in multiple organ systems. 1,2 Approximately 4 to 250 of every 100 000 people are affected by SLE, and women are affected 10 times more frequently than men. 2 At the time of SLE diagnosis, up to 50% of patients have clinical evidence of kidney disease, defined as lupus nephritis (LN). 3 During follow-up, more than 60% of patients will have renal involvement, and approximately one third of patients will require medical treatment for kidney disease. [2][3][4] LN usually develops during the first 3 years following SLE diagnosis or may be the first symptom leading to diagnosis. 2 The prevalence of nephritis in SLE patients varies among ethnicities, occurring in about 17% of Caucasians, 50% of blacks, 43% of Hispanics, and up to 70% to 80% of some Southeast Asian populations. 1,5 Patients with SLE have dysregulated production of antibodies that act against antigens present in the body, which leads to the formation of immune complexes. 2 The hallmark feature of LN is the deposition of immune complexes in the kidneys resulting in glomerular injury. [5][6][7] Immune deposits in kidneys elicit immune reactions. 2 The activation of complement generates chemoattractants and leads to an influx of neutrophils and mononuclear cells causing cellular damage. This can manifest as a proliferative glomerulonephritis with active urine sediment, proteinuria, and decreased renal 500923P MTXXX10.