Involvement of neutrophil extracellular traps in the pathogenesis of glomerulonephritis in a case of systemic lupus erythematosus and antineutrophil cytoplasmic antibody-associated vasculitis overlap syndrome

Abstract

Systemic lupus erythematosus (SLE) and antineutrophil cytoplasmic antibody-associated vasculitis (AAV) are autoimmune diseases that often cause rapidly progressive glomerulonephritis, with neutrophil extracellular traps (NETs) involved in their pathogenesis. However, the involvement of NETs in the renal damage caused by SLE/AAV overlap syndrome has not been clarified yet. In this study, we detected renal deposition of NETs in a patient with SLE/AAV overlap syndrome. In addition, a significantly increased level of NET-inducing activity was observed in the patient’s serum, which improved with treatment. On the other hand, a markedly lower level of NET degradation was observed in the patient’s serum as compared to healthy subjects’ sera, without any posttreatment changes. These findings suggest that NETs may play a role in the pathogenesis of renal injury associated with SLE/AAV overlap syndrome.

Coincidence of Systemic Lupus Erythematosus and ANCA-Associated Vasculitis: A Case Report with Perforation of Nasal Septum and Palate

“…Recently, it has been proposed that neutrophil extracellular traps (NETs) play a significant role in a variety of autoimmune diseases, such as SLE and AAV. 15 NETs are extracellular networks of DNA scaffolds decorated with granular components, histones and cytoplasmic proteins which protrude from the membrane of activated neutrophils. 16 , 17 Impaired degradation of NETs was observed in the serum of SLE patients, which was due to the presence of DNase-1 inhibitors and anti-NET antibodies that prevented access of DNase-1 to NETs.…”