Serum levels of beta2-microglobulin and free light chains of immunoglobulins are associated with systemic disease activity in primary Sjögren's syndrome. Data at enrollment in the prospective ASSESS cohort.
Serum levels of beta2-microglobulin and free light chains of immunoglobulins are associated with systemic disease activity in primary Sjögren's syndrome. Data at enrollment in the prospective ASSESS cohort.
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DOI：
10.1371/journal.pone.0059868
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发表时间：
2013
期刊：
影响因子：
3.7
通讯作者：
Mariette X
中科院分区：
文献类型：
--
作者：
Gottenberg JE;Seror R;Miceli-Richard C;Benessiano J;Devauchelle-Pensec V;Dieude P;Dubost JJ;Fauchais AL;Goeb V;Hachulla E;Hatron PY;Larroche C;Le Guern V;Morel J;Perdriger A;Puéchal X;Rist S;Saraux A;Sene D;Sibilia J;Vittecoq O;Nocturne G;Ravaud P;Mariette X
分析原发性干燥综合征（pSS）患者大型前瞻性队列入组时的临床和免疫学特征，并研究血清BAFF、β 2-微球蛋白和免疫球蛋白游离轻链与入组时全身疾病活动性之间的相关性。根据美国-欧洲共识标准，395例pSS患者来自15个风湿病学和内科中心，被纳入“干燥综合征的全身体征和演变评估”（ASSESS）5年前瞻性队列。在入组时，评估血清标志物以及用EULAR干燥综合征疾病活动指数（ESSDAI）测量的疾病活动。患者中位年龄为58岁（第25 - 75岁：51-67岁），中位病程为5年（2-9年）。入组时的中位ESSDAI为2（0-7），30.9%的患者具有全身受累特征。BAFF、β 2-微球蛋白和κ、λ FLCS升高的患者在入组时ESSDAI评分较高（分别为4 vs 2 [0 -7]，P = 0.03; 4 vs 2 [0-7]，P< 0.0001）; 4 vs 2 [0-6.6]，P< 0.0001和4 [2-8.2] vs 2 [0-7.0]，P = 0.02）。 在多变量分析中，β 2-微球蛋白、κ和λ FLC升高与较高的ESSDAI评分相关。16例有淋巴瘤病史的患者中位BAFF和β 2-微球蛋白较高（分别为1173.3（873.1-3665.5）vs 898.9（715.9-1187.2）pg/ml，P = 0.01和2.6（2.2-2.9）vs 2.1（1.8-2.6）mg/l，P = 0.04）。 在pSS中，较高水平的β 2-微球蛋白和免疫球蛋白游离轻链与全身疾病活动增加相关。
To analyze the clinical and immunological characteristics at enrollment in a large prospective cohort of patients with primary Sjögren's syndrome (pSS) and to investigate the association between serum BAFF, beta2-microglobulin and free light chains of immunoglobulins and systemic disease activity at enrollment. Three hundred and ninety five patients with pSS according to American-European Consensus Criteria were included from fifteen centers of Rheumatology and Internal Medicine in the “Assessment of Systemic Signs and Evolution of Sjögren's Syndrome” (ASSESS) 5-year prospective cohort. At enrollment, serum markers were assessed as well as activity of the disease measured with the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI). Patient median age was 58 (25th–75th: 51–67) and median disease duration was 5 (2–9) years. Median ESSDAI at enrollment was 2 (0–7) with 30.9% of patients having features of systemic involvement. Patients with elevated BAFF, beta2-microglobulin and kappa, lambda FLCS had higher ESSDAI scores at enrollment (4 vs 2 [0–7], P = 0.03; 4 vs 2 [0–7], P< 0.0001); 4 vs 2 [0–6.6], P< 0.0001 and 4 [2–8.2] vs 2 [0–7.0], P = 0.02, respectively). In multivariate analysis, increased beta2-microglobulin, kappa and lambda FLCs were associated with a higher ESSDAI score. Median BAFF and beta2-microglobulin were higher in the 16 patients with history of lymphoma (1173.3(873.1–3665.5) vs 898.9 (715.9–1187.2) pg/ml, P = 0.01 and 2.6 (2.2–2.9) vs 2.1 (1.8–2.6) mg/l, P = 0.04, respectively). In pSS, higher levels of beta2-microglobulin and free light chains of immunoglobulins are associated with increased systemic disease activity.
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没有证据表明-871 T/C启动子多态性在B细胞激活因子基因和原发性Sjögren综合征之间存在关联。
影响因子：
4.9
作者：
Gottenberg, Jacques-Eric;Sellam, Jeremie;Ittah, Marc;Lavie, Frederic;Proust, Alexis;Zouali, Habib;Sordet, Christelle;Sibilia, Jean;Kimberly, Robert P;Mariette, Xavier;Miceli-Richard, Corinne
通讯作者：
Miceli-Richard, Corinne
影响因子：
9.1
作者：
Jonsson, MV;Szodoray, P;Skarstein, K
通讯作者：
Skarstein, K
影响因子：
27.4
作者：
Candon, S.;Gottenberg, J. E.;Mariette, X.
通讯作者：
Mariette, X.
影响因子：
27.4
作者：
Lavie, Frederic;Miceli-Richard, Corinne;Mariette, Xavier
通讯作者：
Mariette, Xavier
影响因子：
158.5
作者：
MICHALSKI, JP;DANIELS, TE;GREY, HM
通讯作者：
GREY, HM