Department of Pathology, Sawai Man Singh Medical College, Jaipur, Rajasthan, India.
Saudi J Kidney Dis Transpl. 2022 Aug;33(Supplement):S255-S259. doi: 10.4103/1319-2442.384195.
Sjogren's syndrome is a prevalent and underdiagnosed systemic disease that primarily affects epithelial tissue. It may affect renal function, either as an epithelial disease causing tubulointerstitial nephritis (TIN) or as an immune complex-mediated glomerulopathy. These lesions may cause a variety of clinical features, both overt and occult. The epithelial disease is mediated by B and T cells. The prevalence of renal involvement in primary Sjogren's syndrome has been reported to range approximately from 10% to 30%. Patients with renal involvement in primary Sjogren's syndrome may have renal manifestations, such as renal tubular acidosis (RTA) Type I, TIN, diabetes insipidus, nephrolithiasis, and Fanconi syndrome. Distal RTA was reported more commonly than proximal RTA, which can present as acute hypokalemic paralysis. We present three cases of primary Sjogren's syndrome with significant renal involvement and various clinicopathological presentations.
干燥综合征是一种常见且易被漏诊的系统性疾病，主要影响上皮组织。它可能会影响肾功能，既可以作为导致肾小管间质性肾炎（TIN）的上皮疾病，也可以作为免疫复合物介导的肾小球病。这些病变可能会导致各种显性和隐匿性的临床特征。上皮疾病由 B 细胞和 T 细胞介导。原发性干燥综合征患者的肾脏受累的发生率据报道约为 10%至 30%。原发性干燥综合征患者的肾脏受累可表现为肾脏管状酸中毒（RTA）I 型、TIN、尿崩症、肾结石和 Fanconi 综合征。远端 RTA 比近端 RTA 更为常见，可表现为急性低钾性瘫痪。我们报告了三例原发性干燥综合征伴显著肾脏受累和多种临床病理表现的病例。
