Abstract
Systemic lupus erythematosus (SLE) is the prototype of autoimmune disorders caused by a loss of tolerance to endogenous nuclear antigens triggering an aberrant autoimmune response targeting various tissues. Lupus nephritis (LN), a major cause of morbidity and mortality in patients with SLE, affects up to 60% of patients. The recent insights into the genetic and molecular basis of SLE and LN paved the way for newer therapies to be developed for these patients. Apart from the traditional B-cell-centered view of this disease pathogenesis, acknowledging that multiple extrarenal and intrarenal pathways contribute to kidney-specific autoimmunity and injury may help refine the individual therapeutic and prognostic characterization of such patients. Accordingly, the formerly induction-maintenance treatment strategy was recently challenged with the exciting results obtained from the trials that evaluated add-on therapy with voclosporin, belimumab, or Obinutuzumab. The scope of this review is to provide an insight into the current knowledge of LN pathogenesis and future therapeutic strategies.
Highlights
Systemic lupus erythematosus (SLE) is the prototype of autoimmune disorders caused by a loss of tolerance to endogenous nuclear antigens triggering an aberrant autoimmune response targeting various tissues [1,2]
SLE and lupus nephritis (LN) pathogenesis clearly involve a genetic predisposition as first-degree relatives of patients with SLE are at higher risk of SLE and other autoimmune disorders [11]
Patients who were deficient in either DNAase I, involved in neutrophil extracellular traps (NETs) degradation, and C1q, essential for NETs opsonization, and clearance, had a continuous source of nuclear autoantigens capable of sustaining the autoimmunity [14]
Summary
Systemic lupus erythematosus (SLE) is the prototype of autoimmune disorders caused by a loss of tolerance to endogenous nuclear antigens triggering an aberrant autoimmune response targeting various tissues [1,2]. We showed that the treatment-related morbidity is substantial, the incidence rate of infections and serious infections being 26.6 and 9.56 events per 100 patient-years, respectively [6] Both patient and renal survival significantly improved over the past decades with the advent of newer treatment strategies [7,8]. After several negative trials that had failed to demonstrate the superiority of the tested drugs over the current standard of care, recent years brought ground-breaking results to the LN treatment landscape [10] This progress was superimposed on a better understanding of the genetic and molecular basis of LN, paving the way for refining and individualizing patient management [1,2].
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