Movva Suneel, Carsons Steven
Division of Rheumatology, Allergy and Immunology, Department of Medicine, Winthrop University Hospital, Mineola, New York.
Pediatr Allergy Immunol Pulmonol. 2014 Sep;27(3):111-114. doi: 10.1089/ped.2013.0326. Epub 2014 May 20.
Sjögren's syndrome is an autoimmune condition characterized by periductal and perivascular lymphocytic infiltration of exocrine glandular ducts, xerostomia, and keratoconjunctivitis sicca. It was first described by Henrik Sjögren and Henri Gougerot in the early 1900's. Since that time, Sjögren's syndrome has been relatively underreported in pediatric literature. Although similar to the disease in the adult population, pediatric Sjögren's most notably presents with less sicca and more parotid swelling. In this chapter, we will describe the unique clinical, serological, and extraglandular manifestations of Sjögren's syndrome in the pediatric population. Furthermore, we will review the diagnostic and therapeutic approach by evaluating the risks and benefits of pursuing an invasive diagnostic workup in a young population, as well as the implications of treatment that can sometimes require long-term immunosuppression.
干燥综合征是一种自身免疫性疾病，其特征为外分泌腺导管周围和血管周围淋巴细胞浸润、口干症和干燥性角结膜炎。它最早由亨里克·舍格伦（Henrik Sjögren）和亨利·古热罗（Henri Gougerot）在20世纪初描述。从那时起，干燥综合征在儿科文献中的报道相对较少。尽管小儿干燥综合征与成人患者的疾病相似，但小儿干燥综合征最显著的表现是口干症状较轻，腮腺肿大较多。在本章中，我们将描述小儿干燥综合征独特的临床、血清学和腺体外表现。此外，我们将通过评估在年轻人群中进行侵入性诊断检查的风险和益处，以及有时需要长期免疫抑制治疗的影响，来回顾其诊断和治疗方法。
