Sjögren综合征(SS)和蛋白丢失性肠病(PLE)之间的关联被严格报道。临床分析,治疗性的,以及SS和PLE患者的结局特征,并描述将肠道与SS靶向器官病理联系起来的潜在机制和途径。使用PubMed/MEDLINE进行系统筛查,LILACS,SciELO,WebofScience,还有Cochrane,从1980年到2020年。SS和PLE是关键词。总结18例SS和PLE患者。病人的年龄在20到88岁之间,只有4个是男性。在大多数情况下观察到原发性SS。在100%的病例中检测到抗Ro,而在64%的病例中报告了抗La。临床表现为蛋白质丢失,下肢水肿,胸腔积液,腹水,面部水肿,Anasarca,腹泻,和减肥。在这些临床表现中,下肢水肿最为严重。白蛋白浓度为0.9-3.4g/dL,处理后增加至2.8-4.3g/dL。所有病例均进行了小肠活检。关于治疗,所有患者均接受全身性糖皮质激素治疗.他们都改进了。起效时间为3周至36个月(平均3个月)。抗Ro阳性SS伴水肿患者PLE的早期诊断和适当治疗,Anasarca,或低蛋白血症对有益的结果至关重要。在所有病例中观察到良好的临床改善时,早期通过皮质治疗,从而防止病人的恶化,并发症,降低发病率和潜在死亡率。
The association between Sjögren\'s syndrome (SS) and protein-losing enteropathy (PLE) was scarcly reported. To analyze the clinical, therapeutic, and outcome characteristics of patients with SS and PLE and also to delineate the potential mechanisms and pathways connecting the gut to SS targeted organ\'s pathology. Systematic screening was conducted using PubMed/MEDLINE, LILACS, SciELO, Web of Science, and Cochrane, dating 1980 to 2020. SS and PLE were the key words. Eighteen patients with SS and PLE were summarized. The patient\'s ages ranged between 20 and 88 years, and only 4 were males. Primary SS was observed in most cases. Anti-Ro was detected in 100% of the cases while anti-La was reported in 64% of them. The clinical manifestations were protein loss, edema of the lower limbs, pleural effusion, ascites, facial edema, anasarca, diarrhea, and weight loss. Among these clinical manifestations, edema of the lower limbs was the most severe. Albumin concentration was 0.9-3.4 g/dL which increased to 2.8-4.3 g/dL after treatment. Small bowel biopsy was performed in all of the cases. Concerning the therapy, all the patients received systemic glucocorticoids. All of them improved. The period of onset of improvement ranged from 3 weeks to 36 months (an average of 3 months). The early diagnosis and appropriate therapy of PLE in patients with anti-Ro positive SS and who present edema, anasarca, or hypoalbuminemia is vital for a beneficial outcome. An excellent clinical improvement in all the cases was observed when treated early enough by cortico-therapy, thus preventing patient\'s deterioration, complications, and reducing morbidity and potential mortality.