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Dysregulated neutrophil responses and neutrophil extracellular trap formation and degradation in PAPA syndrome

Annals of Rheumatic Diseases Aug 26, 2018

Mistry P, et al. - Authors sought to elucidate the potential role of neutrophil subsets and neutrophil extracellular traps (NET) in the pathogenesis of pyoderma gangrenosum and acne (PAPA). An imbalance of NET formation and degradation, that may enhance the half-life of these structures in vivo, thereby promoting inflammation, characterized the PAPA syndrome. In this disease, a role for IL-1 signalling in exacerbated neutrophil responses is supported by anakinra ameliorates NET formation in PAPA. Other inflammatory pathways potentially pathogenic in PAPA, including IL-17 and IL-6 were highlighted in the results. In this severe and often treatment-refractory condition, new therapeutic approaches may be guided by these findings.
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