Department of Endocrinology and Metabolism, Dışkapı Yıldırım Beyazıt Education and Researching Hospital, 06110 Altındağ, Ankara, Turkey.
Rheumatol Int. 2013 Jul;33(7):1879-82. doi: 10.1007/s00296-011-2322-z. Epub 2012 Jan 3.
We report a 53-year-old Turkish female presented with progressive weakness and mild dyspnea. Laboratory results demonstrated severe hypokalemia with hyperchloremic metabolic acidosis. The urinary anion gap was positive in the presence of acidemia, thus she was diagnosed with hypokalemic paralysis from a severe distal renal tubular acidosis (RTA). Immunologic work-up showed a strongly positive ANA of 1:3,200 and positive antibodies to SSA and SSB. Schirmer's test was abnormal. Autoimmune and other tests revealed Sjögren syndrome as the underlying cause of the distal renal tubular acidosis. Renal involvement in Sjogren's syndrome (SS) is not uncommon and may precede sicca complaints. The pathology in most cases is a tubulointerstitial nephritis causing among other things, distal RTA, and, rarely, hypokalemic paralysis. Treatment consists of potassium repletion, alkali therapy, and corticosteroids. Primary SS could be a differential in women with acute weakness and hypokalemia.
我们报告了一例 53 岁土耳其女性，她表现为进行性无力和轻度呼吸困难。实验室结果显示严重低钾血症伴高氯性代谢性酸中毒。尿阴离子间隙在酸中毒存在时呈阳性，因此她被诊断为严重远端肾小管性酸中毒（RTA）引起的低钾性瘫痪。免疫检查显示抗核抗体（ANA）阳性 1:3200，且抗 SSA 和 SSB 抗体阳性。Schirmer 试验异常。自身免疫和其他检查显示干燥综合征（SS）是导致远端肾小管性酸中毒的潜在原因。干燥综合征（SS）的肾脏受累并不少见，可能先于干燥症状出现。大多数情况下，病理是导致远端 RTA 和罕见的低钾性瘫痪的肾小管间质性肾炎。治疗包括补钾、碱化治疗和皮质类固醇。原发性 SS 可能是女性急性无力和低钾血症的鉴别诊断。
