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Mast cells play a key role in autoinflammatory disease development in PSTPIP2-deficient cmo mice (BA7P.141)


 Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory disease characterized by fevers, bone lesions, and skin lesions. A CRMO-like disease develops in cmo mice, which harbor a mutation in proline serine threonine phosphatase-interacting protein 2 (PSTPIP2). Loss of PSTPIP2 in cmo mice has been linked to elevated macrophage and osteoclast activation, and IL-1β-dependent disease progression. Since mast cells were detected in cmo lesions, we have tested the role of mast cells in cmo disease. In diseased cmo mice, increased connective tissue mast cells were observed in both normal skin and within skin lesions. Crossing cmo mice with a transgenic model of mast cell deficiency (Mcpt5-Cre:Rosa26-Stopfl/fl-DTa) resulted in mice (cmo/MC-) that were protected from developing cmo disease, including skin lesions and joint swelling. Fewer osteolytic lesions were also observed in cmo/MC- mice by micro-computed tomography. Within these tissues, cmo/MC- mice had reduced levels of IL-1β, and less activated immune cells in popliteal lymph nodes compared to cmo. Intravital microscopy experiments on young cmo mice revealed increased leukocyte recruitment in response to lipopolysaccharide compared to cmo/MC- and wild-type mice. Together, these results implicate mast cells in promoting leukocyte recruitment to inflamed tissues during autoinflammatory disease progession, and suggest that therapies targeting mast cells or their mediators may benefit CRMO patients.