Khandelwal Deepak, Bhattacharya Saptarshi, Khadgawat Rajesh, Kaur Satbir, Tandon Nikhil, Ammini Ariachery C
Department of Endocrinology and Metabolism, All India Institute of Medical Sciences, New Delhi, India.
Indian J Endocrinol Metab. 2012 Sep;16(5):853-5. doi: 10.4103/2230-8210.100684.
Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease characterized by a progressive lymphocytic infiltration of the exocrine glands with varying degrees of systemic involvement. Overt or latent renal tubular acidosis (RTA), caused by tubulointerstitial nephropathy, is a common extraglandular manifestation of pSS. Hypokalemic paralysis is a well known, albeit rare complication of severe distal RTA from any cause. Cases of pSS manifesting for the first time as hypokalemic paralysis caused by distal RTA have been rarely reported. We herein present our experience of two cases, who presented to us for evaluation of hypokalemic paralysis and on work up found evidence of distal RTA, which on further work up found to be secondary to pSS. A high index of suspicion for pSS should be kept in all patients with hypokalemic paralysis.
原发性干燥综合征（pSS）是一种慢性自身免疫性疾病，其特征是外分泌腺进行性淋巴细胞浸润，并伴有不同程度的全身受累。由肾小管间质性肾病引起的显性或隐性肾小管酸中毒（RTA）是pSS常见的腺体外表现。低钾性麻痹是任何原因导致的严重远端RTA的一种众所周知但罕见的并发症。首次表现为远端RTA所致低钾性麻痹的pSS病例鲜有报道。我们在此介绍两例患者的情况，他们因低钾性麻痹前来就诊，检查发现有远端RTA的证据，进一步检查发现是继发于pSS。对于所有低钾性麻痹患者，都应高度怀疑pSS。
