Primary Sjögren’s syndrome with renal tubular acidosis and central pontine myelinolysis: An unusual triad

Barday, Zunaid; Masikati, Malcolm; Wearne, Nicola; Rayner, Brian; Davidson, Bianca; Bateman, Kathleen; Jones, Erika

doi:10.5414/cncs110994

Cited by 2 publications

(2 citation statements)

References 23 publications

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“…While RTA itself does not typically warrant immunomodulatory therapy in Sjogren's syndrome, despite its status as an extra‐glandular manifestation, 11 previous reports have indicated effective treatment outcomes for RTA in SS using immunosuppressive medications such as Cyclophosphamide. 15 In our case, full recovery was achieved without the use of immunomodulatory medication.…”

Section: Discussionmentioning

confidence: 48%

Hypokalemic quadriplegia in Sjogren's syndrome: A case report

Mehdipour Dalivand,

Abdolazimi,

Alikhani

2024

Clinical Case Reports

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Key Clinical MessageIn 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.AbstractDistal 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.

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Section: Discussionmentioning

confidence: 48%

Hypokalemic quadriplegia in Sjogren's syndrome: A case report

Mehdipour Dalivand,

Abdolazimi,

Alikhani

2024

Clinical Case Reports

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“…Most of these patients had underlying diseases like diabetes mellitus, anorexia nervosa, chronic alcoholism, and hypoparathyroidism [ 12 ]. There are few case reports of patients with SS who developed CPM with hypokalemia and/or hypernatremia [ 13 , 14 , 15 ]. Hypokalemia causing nephrogenic diabetes insipidus, followed by a rapid rise in sodium resulting in osmotic stress, could potentially explain the occurrence of CPM in SS [ 16 ].…”

Section: Discussionmentioning

confidence: 99%

Central Pontine Myelinolysis and Hypokalemic Paralysis as Presenting Manifestations of Sjogren’s Syndrome

V V,

Narayanan,

Bhasi

et al. 2023

Cureus

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Neurological involvement in Sjogren’s syndrome can have varied manifestations and can precede the classical sicca symptoms of Sjogren’s syndrome. A 32-year-old woman presented with acute quadriparesis and dysarthria. She had severe hypokalemia, and an MRI of the brain showed a lesion in the central pons that was hyperintense on T2 and fluid-attenuated inversion recovery (FLAIR) sequences sparing the periphery, a trident appearance characteristic of central pontine myelinolysis (CPM). On further evaluation, she was found to have distal renal tubular acidosis (dRTA) due to primary Sjogren’s syndrome. She was treated with steroids and other supportive measures, and she recovered completely in one month. We describe a mild form of CPM with classical MRI features in a patient with Sjogren’s syndrome and hypokalemia due to dRTA.

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Primary Sjögren’s syndrome with renal tubular acidosis and central pontine myelinolysis: An unusual triad

Cited by 2 publications

References 23 publications

Hypokalemic quadriplegia in Sjogren's syndrome: A case report

Hypokalemic quadriplegia in Sjogren's syndrome: A case report

Central Pontine Myelinolysis and Hypokalemic Paralysis as Presenting Manifestations of Sjogren’s Syndrome

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