Romão Vasco C, Talarico Rosaria, Scirè Carlo Alberto, Vieira Ana, Alexander Tobias, Baldini Chiara, Gottenberg Jacques-Eric, Gruner Heidi, Hachulla Eric, Mouthon Luc, Orlandi Martina, Pamfil Cristina, Pineton de Chambrun Marc, Taglietti Marco, Toplak Natasa, van Daele Paul, van Laar Jacob M, Bombardieri Stefano, Schneider Matthias, Smith Vanessa, Cutolo Maurizio, Mosca Marta, Mariette Xavier
Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon, Portugal.
Rheumatology Unit, AOU Pisana, Pisa, Italy.
RMD Open. 2018 Oct 18;4(Suppl 1):e000789. doi: 10.1136/rmdopen-2018-000789. eCollection 2018.
Sjögren's syndrome (SS) is a complex autoimmune rheumatic disease that specifically targets salivary and lachrymal glands. As such, patients typically had ocular and oral dryness and salivary gland swelling. Moreover, skin, nasal and vaginal dryness are frequently present. In addition to dryness, musculoskeletal pain and fatigue are the hallmarks of this disease and constitute the classic symptom triad presented by the vast majority of patients. Up to 30% to 50 % of patients with SS may present systemic disease; moreover, there is an increased risk for the development of non-Hodgkin's lymphoma that occurs in a minority of patients. The present work was developed in the framework of the European Reference Network (ERN) dedicated to Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET). In line with its goals of aiming to improve early diagnosis, treatment and care of rare connective and musculoskeletal diseases, ERN-ReCONNET set to review the current state of clinical practice guidelines (CPGs) in the rare and complex connective tissue diseases of interest of the network. Therefore, the present work was aimed at providing a state of the art of CPGs for SS.
干燥综合征（SS）是一种复杂的自身免疫性风湿性疾病，主要累及唾液腺和泪腺。因此，患者通常有眼干、口干以及唾液腺肿大的症状。此外，皮肤、鼻腔和阴道干燥也较为常见。除了干燥症状外，肌肉骨骼疼痛和疲劳是该疾病的特征，也是绝大多数患者呈现的典型三联征症状。高达30%至50%的干燥综合征患者可能出现全身性疾病；此外，少数患者患非霍奇金淋巴瘤的风险增加。本研究是在欧洲罕见和复杂结缔组织及肌肉骨骼疾病参考网络（ERN）（ReCONNET）的框架内开展的。为了实现改善罕见结缔组织和肌肉骨骼疾病的早期诊断、治疗和护理的目标，ERN-ReCONNET着手审查网络所关注的罕见和复杂结缔组织疾病的临床实践指南（CPG）的现状。因此，本研究旨在提供干燥综合征CPG的最新情况。
