Varma Vansh, Patel Ajay Kumar, Pathak Nitya, Patel Abhishek, Kumar Shubham, Gaidhane Shilpa, Sah Sanjit, Satapathy Prakasini, Mehta Rachana, Verma Amogh
GMERS Medical College and Hospital, Valsad, India.
GMERS Medical College and Hospital, Gotri, India.
Clin Med Insights Case Rep. 2025 Aug 30;18:11795476251372407. doi: 10.1177/11795476251372407. eCollection 2025.
Hypokalemic periodic paralysis (HPP) is a severe yet reversible neuromuscular condition precipitated by profound hypokalemia. Autoimmune disorders can exacerbate renal potassium loss resulting in abrupt muscle weakness. Primary Sjögren's syndrome (pSS), an autoimmune disease characterized by exocrine gland insufficiency, can lead to renal tubular dysfunction and episodes of HPP when distal acidification is compromised.
A 40-year-old woman was admitted with rapidly progressive, painless quadriplegia for over 2 days. Laboratory tests revealed critical hypokalemia (1.4 mEq/L), metabolic acidosis, and alkaline urine pH, which was consistent with type 1 distal renal tubular acidosis (dRTA). Serologic studies confirmed pSS. Corrective measures included intravenous potassium chloride and sodium bicarbonate along with immunomodulation with intravenous methylprednisolone, followed by oral prednisolone.
The patient's presentation illustrates how autoimmune-mediated renal tubular dysfunction can precipitate HPP. Failure of distal acid excretion impairs potassium handling, amplifying the risk of potentially life-threatening neuromuscular collapse. Stabilization requires meticulous electrolyte repletion and treatment of the underlying autoimmunity. Restoration of serum potassium levels, acid-base balance, and targeted immunosuppression resulted in rapid clinical improvement.
An accurate diagnosis of HPP secondary to dRTA and Sjögren's syndrome requires high clinical suspicion. Prompt recognition and intervention, including immunotherapy and balanced electrolyte replacement, can prevent profound neuromuscular complications and improve patient outcome.
低钾性周期性麻痹（HPP）是一种严重但可逆的神经肌肉疾病，由严重低钾血症引发。自身免疫性疾病可加剧肾脏钾流失，导致突然的肌肉无力。原发性干燥综合征（pSS）是一种以外分泌腺功能不全为特征的自身免疫性疾病，当远端酸化功能受损时，可导致肾小管功能障碍和HPP发作。
一名40岁女性因快速进展的无痛性四肢瘫痪入院超过2天。实验室检查显示严重低钾血症（1.4 mEq/L）、代谢性酸中毒和碱性尿pH值，这与1型远端肾小管酸中毒（dRTA）一致。血清学研究证实为pSS。纠正措施包括静脉注射氯化钾和碳酸氢钠，以及静脉注射甲泼尼龙进行免疫调节，随后口服泼尼松龙。
患者的表现说明了自身免疫介导的肾小管功能障碍如何引发HPP。远端酸排泄功能障碍会损害钾的处理，增加潜在危及生命的神经肌肉崩溃的风险。病情稳定需要精心补充电解质并治疗潜在的自身免疫性疾病。血清钾水平、酸碱平衡的恢复以及针对性的免疫抑制导致了临床快速改善。
准确诊断由dRTA和干燥综合征继发的HPP需要高度的临床怀疑。及时识别和干预，包括免疫治疗和平衡的电解质替代，可以预防严重的神经肌肉并发症并改善患者预后。
