Figure 5.

Flux of blood cells and serum cytokines in PFAPA. Proposed oscillations in the abundance of blood cell populations and inflammatory cytokines over time (x-axis: h, hours; d, days; wk, weeks) in a typical PFAPA cycle. Pro-inflammatory cytokines in the serum (TNFα, IL1β, IFNγ, IL6) appear early after the onset of fever [22] and are maintained (IL6) or rapidly decline (TNFα, IL1β, IFNγ) as IFNγ-induced chemokines (MIG, IP10) appear. Lymphocyte activators IL7 and IL17 may be constitutively suppressed. The febrile period is associated with increased neutrophils (PMN) and monocytes (Mo) as well as decreased lymphocytes (Lym). Eosinophils (Eos) are in sparse abundance in both febrile and afebrile intervals while thrombocytes (Thr) rise specifically in the afebrile period. * indicates the onset of fever.