PDF(Pubmed)
Abstract：
Juvenile primary Sjögren syndrome (pSS) with renal involvement is extremely rare, reported approximately in 50 children, predominantly girls. Here, we present the first reported case of a male child with juvenile pSS with ocular surface disease (previously keratoconjunctivitis sicca), submandibular salivary gland involvement, and tubulointerstitial nephritis. First, two symptoms were clinically apparent at presentation. We illustrate here that kidney involvement in pSS should be actively looked for, as juvenile pSS may be associated with asymptomatic renal involvement. Immunophenotyping of peripheral blood cells using multicolor flow cytometry revealed at the time of diagnosis changes in both adaptive (T memory cells and B memory cells), and innate immunity (an increased activation of natural killer cells, as well as monocytes and neutrophils, and an increased representation of intermediate monocytes). Our case report points to the importance of kidney examination, early diagnosis and therapy in juvenile pSS, as well as highlights international collaboration to obtain more data for this rare disease.
摘要：
肾脏受累的青少年原发性干燥综合征（pSS）极为罕见，报告了大约50名儿童，主要是女孩。这里,我们介绍了第一例报道的男性儿童患有青少年pSS眼表疾病（以前是干燥性角膜结膜炎），颌下腺受累，和肾小管间质性肾炎。首先,两种症状在临床上表现明显.我们在这里说明应该积极寻找肾脏参与pSS，因为青少年pSS可能与无症状的肾脏受累有关。使用多色流式细胞术对外周血细胞进行免疫表型分型，在诊断时显示了适应性（T记忆细胞和B记忆细胞）的变化，和先天免疫（自然杀伤细胞的激活增加，以及单核细胞和中性粒细胞，和中间单核细胞的代表增加)。我们的病例报告指出了肾脏检查的重要性，青少年pSS的早期诊断和治疗，以及强调国际合作以获得这种罕见疾病的更多数据。
