Brito-Zerón Pilar, Ramos-Casals Manuel, Nardi Norma, Cervera Ricard, Yagüe Jordi, Ingelmo Miguel, Font Josep
From the Departments of Autoimmune Diseases (PB-Z, MR-C, NN, RC, MI, JF) and Immunology (JY), Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Hospital Clínic, Facultad de Medicina, Universitat de Barcelona, Barcelona, Spain.
Medicine (Baltimore). 2005 Mar;84(2):90-97. doi: 10.1097/01.md.0000157398.37679.47.
We conducted the current study to analyze the prevalence and clinical significance of circulating monoclonal immunoglobulins in patients with Sjögren syndrome (SS), focusing on the association with extraglandular features, immunologic markers, hematologic neoplasia, and hepatitis C virus (HCV) infection. We performed serum immunoelectrophoresis in 200 patients with primary SS and 37 patients with HCV-related SS. All patients fulfilled 4 or more of the 1993 European classification criteria for SS.Of the 200 patients with primary SS, 35 (18%) presented circulating monoclonal immunoglobulins. The monoclonal bands identified were 20 IgG (13 kappa, 7 lambda), 10 IgM (5 kappa, 5 lambda), 2 IgAkappa, and 3 free circulating light chains. Of the 37 SS-HCV patients, 16 (43%) had circulating monoclonal immunoglobulins. The monoclonal bands identified were 10 IgMkappa, 5 IgGlambda, and 1 free light lambda chain. Compared with primary SS patients, SS-HCV patients presented a higher frequency of monoclonal immunoglobulins (43% vs 18%, p = 0.001), with monoclonal IgMkappa being the most frequent monoclonal band. Six (12%) of the 51 SS patients with circulating monoclonal immunoglobulins presented hematologic neoplasia, compared with 3 (1.6%) of those without monoclonal immunoglobulins (p = 0.004; odds ratio = 8.13; 95% confidence intervals, 1.64-51.54). In 2 of the 6 patients with monoclonal immunoglobulins and lymphoproliferative disorders, a change of the monoclonal component was detected in previous immunoelectrophoresis determinations before the development of hematologic neoplasia. Circulating monoclonal immunoglobulins were detected in nearly 20% of patients with primary SS, with monoclonal IgG being the most frequent type of immunoglobulin detected. In SS-HCV patients, the prevalence of monoclonal immunoglobulins was higher (43%), with monoclonal IgM being the most frequent type found. SS-HCV patients presented a more restrictive monoclonal expression (limited to either monoclonal IgMkappa or monoclonal IgGlambda) than primary SS patients, who showed all types of heavy and light chains.
我们开展了本研究，以分析干燥综合征（SS）患者循环单克隆免疫球蛋白的患病率及临床意义，重点关注其与腺外表现、免疫标志物、血液系统肿瘤及丙型肝炎病毒（HCV）感染的关联。我们对200例原发性SS患者和37例HCV相关SS患者进行了血清免疫电泳。所有患者均符合1993年欧洲SS分类标准中的4项或更多项标准。在200例原发性SS患者中，35例（18%）出现循环单克隆免疫球蛋白。鉴定出的单克隆条带为20例IgG（13例κ型，7例λ型）、10例IgM（5例κ型，5例λ型）、2例IgAκ型和3例游离循环轻链。在37例SS-HCV患者中，16例（43%）有循环单克隆免疫球蛋白。鉴定出的单克隆条带为10例IgMκ型、5例IgGλ型和1例游离轻链λ型。与原发性SS患者相比，SS-HCV患者单克隆免疫球蛋白的频率更高（43%对18%，p = 0.001），单克隆IgMκ型是最常见的单克隆条带。在51例有循环单克隆免疫球蛋白的SS患者中，6例（12%）出现血液系统肿瘤，而在无单克隆免疫球蛋白的患者中为3例（1.6%）（p = 0.004；比值比 = 8.13；95%置信区间，1.64 - 51.54）。在6例有单克隆免疫球蛋白和淋巴增殖性疾病的患者中，2例在血液系统肿瘤发生前的既往免疫电泳测定中检测到单克隆成分的变化。在近20%的原发性SS患者中检测到循环单克隆免疫球蛋白，其中单克隆IgG是检测到的最常见免疫球蛋白类型。在SS-HCV患者中，单克隆免疫球蛋白的患病率更高（43%），单克隆IgM是最常见类型。与原发性SS患者显示所有类型的重链和轻链不同，SS-HCV患者的单克隆表达更具局限性（仅限于单克隆IgMκ型或单克隆IgGλ型）。
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