Chikanza Ian, Akpenyi Onyinye
Department of Rheumatology, St Barts & The Royal London Hospital, London, United Kingdom.
Mediterr J Rheumatol. 2018 Jun 29;29(2):80-85. doi: 10.31138/mjr.29.2.80. eCollection 2018 Jun.
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.
已有报道称多种慢性炎症性风湿性疾病与意义未明的单克隆丙种球蛋白病(MGUS)之间存在关联。迄今为止,尚无白塞病(BD)与MGUS相关病例的记录。BD处于自身炎症性疾病和慢性自身免疫性疾病谱的交界位置。细胞和细胞因子微环境的改变可促进促炎状态,在这种状态下,持续的抗原刺激和细胞增殖可逐渐诱导细胞遗传学异常,进而可能扰乱浆细胞功能,如MGUS中所见。在此,我们报告一例罕见病例,一名患有BD的女性随后发展为MGUS。本文回顾了可能导致这两种疾病发生的发病机制,证明这种疾病关联并非偶然,而是由共同的疾病发病机制驱动的一种进化关联。