Rajagopala Srinivas, Danigeti Gurukiran, Subrahmanyan Dharanipragada
Department of Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Dhanvantri Nagar, Puducherry, India.
Indian J Crit Care Med. 2015 Sep;19(9):550-3. doi: 10.4103/0972-5229.164808.
We present a middle-aged woman with a prior history of central nervous system (CNS) demyelinating disorder who presented with an acute onset quadriparesis and respiratory failure. The evaluation revealed distal renal tubular acidosis with hypokalemia and medullary nephrocalcinosis. Weakness persisted despite potassium correction, and ongoing evaluation confirmed recurrent CNS and long-segment spinal cord demyelination with anti-aquaporin-4 antibodies. There was no history of dry eyes or dry mouth. Anti-Sjogren's syndrome A antigen antibodies were elevated, and there was reduced salivary flow on scintigraphy. Coexistent antiphospholipid antibody syndrome with inferior vena cava thrombosis was also found on evaluation. The index patient highlights several rare manifestations of primary Sjogren's syndrome (pSS) as the presenting features and highlights the differential diagnosis of the clinical syndromes in which pSS should be considered in the Intensive Care Unit.
我们报告一名中年女性，既往有中枢神经系统（CNS）脱髓鞘疾病史，此次因急性起病的四肢瘫和呼吸衰竭就诊。评估发现远端肾小管酸中毒伴低钾血症和髓质肾钙质沉着症。尽管纠正了钾缺乏，但肌无力仍持续存在，进一步评估证实存在抗水通道蛋白4抗体阳性的复发性CNS和长节段脊髓脱髓鞘。患者无干眼或口干病史。抗干燥综合征A抗原抗体升高，唾液腺闪烁扫描显示唾液分泌减少。评估还发现并存抗磷脂抗体综合征伴下腔静脉血栓形成。该病例突出了原发性干燥综合征（pSS）的几种罕见表现作为首发特征，并强调了在重症监护病房中应考虑pSS的临床综合征的鉴别诊断。
