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Fig. 7 | Inflammation and Regeneration

Fig. 7

From: Clinical perspectives and therapeutic strategies: pediatric autoinflammatory disease—a multi-faceted approach to fever of unknown origin of childhood

Fig. 7

Schematic view of FMF and diseases with high serum IL-18. Schematic views of FMF and periodic fever syndromes (A) and sJIA/AOSD and related diseases (B) are shown. Typical cases of FMF (orange) consist of cases with characteristic clinical presentation, colchicine response, high serum IL-18, and pathogenic mutations within exon 10 of MEFV (red). Some cases of periodic fever syndromes may also respond to colchicine without exon 10 mutation of MEFV (yellow). These cases, with (dark green) or without mutations (ivory) of MEFV, are classified as atypical FMF. Other than FMF, sJIA, AOSD (blue), and several monogenic diseases, such as XIAP, NLRC4 (violet), and pHLH (navy) are characterized by extremely elevated serum IL-18 and they are prone to develop MAS

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Inflammation and Regeneration

ISSN: 1880-8190

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