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An association between a number of chronic inflammatory rheumatic diseases and Monoclonal Gammopathy of Undetermined Significance (MGUS) has been reported. To date no cases of Behcet’s disease (BD) and MGUS have been documented. BD sits at the interphase of auto-inflammatory and chronic auto-immune disease spectrums. Alterations in the cellular and cytokine microenvironments can promote a pro-inflammatory state in which persistent antigenic stimulation and cellular proliferation can progressively induce cytogenetic abnormalities which could perturbate plasma cell functions such as seen in MGUS. Herein, we present a rare case of a woman presenting with BD who subsequently developed MGUS. Pathogenetic mechanisms that could potentially contribute to development of both conditions, are reviewed and demonstrate that this disease association is not coincidental but is an evolutionary association driven by shared common disease pathogenetic mechanisms.

https://doi.org/10.31138/mjr.29.2.80