Increased pro-inflammatory cytokines (IL)-6, tumor necrosis factor (TNF)-α, interferons (IFNs), and chemokines (CXC, CC, C, and CX3C) are likely related to the extensive systemic inflammation and more severe multi-organ involvement (Table 1).[39,40] Multiple risk factors have been suggested to increase the morbidity and mortality and are linked to worsening outcome and multiorgan involvement such as advanced age, prior medical illness, and obesity.[19,41–43]. This evidence concerns the gene CXCR1 and obesity due to melanocortin 4 receptor deficiency.