Maślińska Maria, Przygodzka Małgorzata, Kwiatkowska Brygida, Sikorska-Siudek Katarzyna
Clinic of Early Arthritis, Institute of Rheumatology, Spartańska 1, 02-637, Warsaw, Poland,
Rheumatol Int. 2015 Feb;35(2):233-41. doi: 10.1007/s00296-014-3072-5. Epub 2014 Jul 2.
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.
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。原发性干燥综合征的发病机制尚不清楚；然而，我们对导致自身免疫发展的不同细胞（如B细胞和T细胞、巨噬细胞和树突状细胞）和途径（BAFF/APRIL和干扰素）的参与情况的了解正在不断扩展。对临床医生来说，最常见的症状是眼睛和口腔干燥，但患者通常还会有肌肉骨骼症状和全身表现。然而，这组患者发生淋巴增殖性疾病的风险增加，最常见的是B细胞边缘区淋巴瘤，这一点尤为重要。最近将IgG4相关疾病分离出来，并尝试为原发性干燥综合征制定进一步的诊断标准，这证明了在理解该疾病以便进行有效治疗方面存在困难，同时也引起了人们的极大兴趣。本文提请注意临床医生在希望通过使用准确的实验室生物标志物和有用的成像工具来可靠地识别原发性干燥综合征，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时所面临的问题。
