Background: Neuro-Sweet disease (NSD) has recently been identified as Sweet disease with central nervous system (CNS) involvement characterized by multisystem neutrophilic infiltration. However, the pathogenesis of this disease remains unknown. Neutrophil and other inflammatory cell activities are influenced by many cytokines and chemokines, but to date, no studies have examined the levels of these factors in patients with NSD.
Patient and methods: The patient presented with encephalomeningitis twice in one year and was diagnosed with NSD. We measured the levels of cytokines (i.e., IL-2, IL-4, IL-6, IL-10, IFN-gamma, and TNF-alpha) and chemokines (i.e., CCL2, CCL3, CCL5, CXCL8, CXCL10 and GM-CSF) in 10 CSF samples from the patient longitudinally for one year including those during two episodes of encephalomeningitis.
Results: The elevations of IL-6, IFN-gamma, CXCL8 (IL8) and CXCL10 (IP10) were markedly higher than the levels in uninfected control subjects with neurological disorders. The levels of these cytokines and chemokines were statistically correlated with total CSF cell counts (p <0.01).
Conclusion: CD4+ helper T (Th) cells can be divided into the Th1 and Th2 subtypes according to their cytokine secretion patterns, and IFN-gamma and IP10 are the Th1-type cytokine and chemokine indicating the involvement of Th1 cells in NSD. In addition, the level of IL8, a specific neutrophil chemoattractant, correlated well with the neutrophil cell counts in CSF. Our data suggest the important roles of Th1 cells and IL8 in the pathogenesis of NSD.