Mehdipour Dalivand Mahsa, Abdolazimi Rezvan, Alikhani Majid
Guilan Rheumatology Research Center, Department of Rheumatology, Razi Hospital, School of Medicine Guilan University of Medical Sciences Rasht Iran.
Rheumatology Research Center Tehran University of Medical Sciences Rasht Iran.
Clin Case Rep. 2024 Jul 31;12(8):e9227. doi: 10.1002/ccr3.9227. eCollection 2024 Aug.
In managing Sjogren's syndrome, a thorough patient history, proper lab tests, and imaging are crucial. Clinicians should prioritize checking electrolyte levels in cases of muscle weakness, as early detection of hypokalemia can prevent severe complications. Proactive monitoring can avert renal tubular acidosis and improve patient outcomes.
Distal renal tubular acidosis (dRTA) occurs in approximately one-third of patients with Sjogren's syndrome, a systemic autoimmune disorder characterized by lymphocytic infiltration of exocrine glands, leading to dryness of mucous membranes. Hypokalemic paralysis, a well-documented but rare complication of dRTA, typically manifests as symmetric proximal muscle weakness of the extremities. We present the case of a 38-year-old woman with a history of Sjogren's syndrome diagnosed 3 years prior, who ceased her medication without medical supervision. She presented with quadriplegia, initially beginning unilaterally. This particular presentation is seldom documented in the literature. Laboratory investigations revealed hypokalemia and normal anion gap metabolic acidosis, consistent with dRTA-induced hypokalemic paralysis. Intravenous potassium chloride was administered, resulting in complete recovery of muscle strength. Hypokalemic paralysis associated with dRTA is typically reversible; however, delays in diagnosis and treatment can lead to life-threatening complications such as respiratory failure and arrhythmias. Therefore, clinicians should maintain a high index of suspicion for this condition in patients presenting with muscle weakness. Prompt and precise history takingand screening, and initiating appropriate management to prevent adverse outcomes.
在干燥综合征的管理中,全面的患者病史、适当的实验室检查和影像学检查至关重要。对于出现肌肉无力的病例,临床医生应优先检查电解质水平,因为早期发现低钾血症可预防严重并发症。积极监测可避免肾小管酸中毒并改善患者预后。
远端肾小管酸中毒(dRTA)发生在约三分之一的干燥综合征患者中,干燥综合征是一种全身性自身免疫性疾病,其特征是外分泌腺淋巴细胞浸润,导致黏膜干燥。低钾性麻痹是dRTA一种有充分文献记载但罕见的并发症,通常表现为四肢对称性近端肌肉无力。我们报告了一例38岁女性病例,她在3年前被诊断为干燥综合征,在未经医学监督的情况下自行停药。她出现四肢瘫痪,最初是单侧起病。这种特殊表现很少在文献中记载。实验室检查显示低钾血症和正常阴离子间隙代谢性酸中毒,符合dRTA引起的低钾性麻痹。静脉输注氯化钾后,肌肉力量完全恢复。与dRTA相关的低钾性麻痹通常是可逆的;然而,诊断和治疗的延迟可能导致危及生命的并发症,如呼吸衰竭和心律失常。因此,临床医生对出现肌肉无力的患者应保持高度的怀疑指数。及时、准确地采集病史和进行筛查,并启动适当的管理措施以预防不良后果。