Sjögren's syndrome: still not fully understood disease.
Sjögren's syndrome: still not fully understood disease.
复制标题
DOI：
10.1007/s00296-014-3072-5
复制
发表时间：
2015-02
影响因子：
4
通讯作者：
Sikorska-Siudek, Katarzyna
中科院分区：
文献类型：
--
作者：
Maslinska, Maria;Przygodzka, Magorzata;Kwiatkowska, Brygida;Sikorska-Siudek, Katarzyna
原发性干燥综合征是一种自身免疫性疾病，伴有外分泌腺功能障碍和多器官受累。原发干燥综合征的发病机制尚不清楚，然而，我们对不同细胞(如B和T细胞、巨噬细胞和树突状细胞)以及导致自身免疫发展的途径(BAFF/APRIL和干扰素)的了解正在不断扩大。对于临床医生来说，最常见的症状是眼睛和口腔干燥，但患者往往有肌肉骨骼症状和全身症状。然而，在这类患者中，淋巴增生性疾病的风险增加，最常见的是B细胞边缘区淋巴瘤，这一点尤其重要。最近对IgG4相关疾病的分离以及为PSS制定进一步诊断标准的尝试证明了理解这种疾病以进行有效治疗的困难，同时也证明了人们的极大兴趣。本文提请注意临床医生所面临的问题，希望通过使用准确的实验室生物标记物和有用的成像工具来安全地识别PSS，并预测与这种仍未完全了解的自身免疫性疾病相关的并发症的发展。
Primary Sjögren's syndrome is an autoimmune disorder with external exocrine glands dysfunction and multiorgan involvement. The pathogenesis of primary Sjogren’s syndrome is still unclear; however, our knowledge of the involvement of different cells (e.g., B and T cells, macrophages and dendritic cells) and pathways (BAFF/APRIL and interferons) leading to the development of autoimmunity is continually expanding. For clinicians, the most frequent symptoms are dryness of eyes and mouth, but often the patients have musculoskeletal symptoms and systemic manifestations. However, the increased risk of lymphoproliferative disorders in this group of patients, most commonly B-cell marginal zone lymphoma, is particularly important. Recent separation of IgG4-related diseases and attempts to create further diagnostic criteria for pSS testify to the difficulties, and at the same time a large interest, in understanding the disease so as to allow the effective treatment. This article draws attention to the problems faced by the clinician wishing to securely identify pSS by using accurate laboratory biomarkers and useful imaging tools and predict the development of complications associated with this, still not fully understood, autoimmune disease.
登录
查看更多内容
影响因子：
27.4
作者：
Rasmussen A;Ice JA;Li H;Grundahl K;Kelly JA;Radfar L;Stone DU;Hefner KS;Anaya JM;Rohrer M;Gopalakrishnan R;Houston GD;Lewis DM;Chodosh J;Harley JB;Hughes P;Maier-Moore JS;Montgomery CG;Rhodus NL;Farris AD;Segal BM;Jonsson R;Lessard CJ;Scofield RH;Sivils KL
通讯作者：
Sivils KL
影响因子：
4.4
作者：
Bohnhorst, JO;Bjorgan, MB;Thompson, KM
通讯作者：
Thompson, KM
影响因子：
12.8
作者：
Quartuccio, Luca;Isola, Miriam;De Vita, Salvatore
通讯作者：
De Vita, Salvatore
影响因子：
1.6
作者：
Ramos-Casals, M;Loustaud-Ratti, V;Font, J
通讯作者：
Font, J
影响因子：
5.5
作者：
Hocevar, A;Ambrozic, A;Tomsic, M
通讯作者：
Tomsic, M