PDF(Pubmed)
Abstract：
Monoclonal gammopathy of clinical significance (MGCS) represents a new clinical entity referring to a myriad of pathological conditions associated with the monoclonal gammopathy of undetermined significance (MGUS). The establishment of MGCS expands our current understanding of the pathophysiology of a range of diseases, in which the M protein is often found. Aside from the kidney, the three main organ systems most affected by monoclonal gammopathy include the peripheral nervous system, skin, and eye. The optimal management of these MGUS-related conditions is not known yet due to the paucity of clinical data, the rarity of some syndromes, and limited awareness among healthcare professionals. Currently, two main treatment approaches exist. The first one resembles the now-established therapeutic strategy for monoclonal gammopathy of renal significance (MGRS), in which chemotherapy with anti-myeloma agents is used to target clonal lesion that is thought to be the culprit of the complex clinical presentation. The second approach includes various systemic immunomodulatory or immunosuppressive options, including intravenous immunoglobulins, corticosteroids, or biological agents. Although some conditions of the MGCS spectrum can be effectively managed with therapies aiming at the etiology or pathogenesis of the disease, evidence regarding other pathologies is severely limited to individual patient data from case reports or series. Future research should pursue filling the gap in knowledge and finding the optimal treatment for this novel clinical category.
摘要：
临床意义的单克隆丙种球蛋白病（MGCS）代表了一种新的临床实体，涉及与不确定意义的单克隆丙种球蛋白病（MGUS）相关的无数病理状况。MGCS的建立扩展了我们目前对一系列疾病的病理生理学的理解，其中经常发现M蛋白。除了肾脏,受单克隆丙种球蛋白病影响最大的三个主要器官系统包括周围神经系统，皮肤,和眼睛。由于缺乏临床数据，尚不知道这些MGUS相关疾病的最佳管理。一些综合症的罕见，以及医疗保健专业人员的意识有限。目前,存在两种主要的治疗方法。第一个类似于现在建立的肾脏意义的单克隆丙种球蛋白病（MGRS）的治疗策略，其中抗骨髓瘤药物的化疗用于靶向克隆性病变，该病变被认为是复杂临床表现的罪魁祸首。第二种方法包括各种全身免疫调节或免疫抑制选择，包括静脉注射免疫球蛋白,皮质类固醇,或生物制剂。尽管MGCS谱的一些病症可以通过针对疾病的病因或发病机理的疗法有效地管理。关于其他病理的证据严重限于病例报告或系列的个体患者数据.未来的研究应该寻求填补知识空白，并为这一新的临床类别找到最佳治疗方法。
