Islam Mohammad Syedul, Mamtaz Uddin Ahmed Quazi, Ahmed Farzana, Uddin Md Ashraf, Naher Naznin
Department of Internal Medicine, Bangabandhu Sheikh Mujib Medical University, Dhaka, BGD.
Department of Pediatrics, Marks Medical College and Hospital, Dhaka, BGD.
Cureus. 2025 Apr 13;17(4):e82206. doi: 10.7759/cureus.82206. eCollection 2025 Apr.
Primary Sjögren's syndrome (pSS) is typically associated with dryness of the eyes and mouth, but it can also involve other organs, including the lungs, kidneys, nervous system, and joints. Among its less common manifestations is distal renal tubular acidosis (dRTA), which can lead to metabolic acidosis, hypokalemia, and bone-related complications due to chronic acid-base imbalance. We report the case of a 42-year-old woman with a four-year history of recurrent hypokalemic quadriparesis, who recently developed progressive difficulty walking over the past two months, severely limiting her mobility. Laboratory investigations revealed a normal anion gap metabolic acidosis and elevated urine pH, consistent with dRTA. Further evaluation confirmed a diagnosis of pSS with objective evidence of glandular involvement. Imaging and biochemical findings supported the presence of osteomalacia secondary to dRTA. This case highlights a rare and often overlooked complication of pSS. Timely diagnosis and appropriate management are crucial to preventing long-term disability and improving patient outcomes.
原发性干燥综合征（pSS）通常与眼干和口干有关，但也可能累及其他器官，包括肺、肾、神经系统和关节。其较不常见的表现之一是远端肾小管酸中毒（dRTA），由于慢性酸碱失衡，可导致代谢性酸中毒、低钾血症和骨相关并发症。我们报告一例42岁女性，有四年复发性低钾性四肢无力病史，最近在过去两个月出现进行性行走困难，严重限制了她的活动能力。实验室检查显示阴离子间隙正常的代谢性酸中毒和尿pH升高，符合dRTA。进一步评估确诊为pSS，并有腺体受累的客观证据。影像学和生化检查结果支持dRTA继发骨软化症的存在。该病例突出了pSS一种罕见且常被忽视的并发症。及时诊断和适当治疗对于预防长期残疾和改善患者预后至关重要。
