Marinkovic Angelina, Zypchen Leslie N, Chan Jonathan, Chen Luke Yc, Parkin Stephen
Department of Medicine, University of British Columbia, Vancouver, BC, Canada.
Division of Hematology, University of British Columbia, Vancouver, BC, Canada.
Lancet Rheumatol. 2022 May;4(5):e362-e373. doi: 10.1016/S2665-9913(21)00348-9. Epub 2022 Feb 15.
Monoclonal proteins can provide important information on the diagnosis of several non-malignant systemic inflammatory disorders. At low concentration, they most commonly represent monoclonal gammopathy of undetermined significance (MGUS), whereas high concentrations often signify plasma cell myeloma or B-cell lymphoma. However, several rare inflammatory conditions associated with variable concentrations of monoclonal proteins, systemic symptoms, and organ dysfunction also exist. These conditions are termed monoclonal gammopathies of clinical significance (MGCS). Patients with MGCS might present to rheumatologists with undiagnosed systemic inflammatory disorders and the monoclonal protein provides an important, underappreciated clue for diagnosis. In this Review, we provide an approach to distinguishing MGCS from MGUS and lymphoid neoplasms, focusing on four rare MGCS that rheumatologists must recognise: scleromyxedema, Schnitzler's syndrome, idiopathic systemic capillary leak syndrome (also known as Clarkson's disease), and telangiectasias, elevated erythropoietin and erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting (known as TEMPI) syndrome.
单克隆蛋白可为多种非恶性系统性炎症性疾病的诊断提供重要信息。在低浓度时，它们最常见的表现是意义未明的单克隆丙种球蛋白病（MGUS），而高浓度通常提示浆细胞骨髓瘤或B细胞淋巴瘤。然而，也存在几种与单克隆蛋白浓度可变、全身症状和器官功能障碍相关的罕见炎症性疾病。这些疾病被称为具有临床意义的单克隆丙种球蛋白病（MGCS）。MGCS患者可能因未确诊的系统性炎症性疾病就诊于风湿病学家处，而单克隆蛋白为诊断提供了一个重要但未得到充分重视的线索。在本综述中，我们提供了一种区分MGCS与MGUS和淋巴样肿瘤的方法，重点关注风湿病学家必须认识的四种罕见MGCS：硬化性黏液水肿、施尼茨勒综合征、特发性系统性毛细血管渗漏综合征（也称为克拉克森病），以及毛细血管扩张、促红细胞生成素升高和红细胞增多、单克隆丙种球蛋白病、肾周液体积聚和肺内分流（称为TEMPI）综合征。
