Recurrent Attacks of Hypokalemic Quadriparesis: An Unusual Presentation of Primary Sjögren Syndrome

Seirafian, Shiva; Shafie, Mohammad; Abedini, Amin; Pakzad, Bahram; Roomizadeh, Peyman

doi:10.2169/internalmedicine.55.6453

Cited by 6 publications

(7 citation statements)

References 19 publications

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“…The two common forms of renal involvement in SS are interstitial nephritis and distal renal tubular acidosis. Other rare renal manifestations include glomerular diseases like membranoproliferative glomerulonephritis (MPGN) and membranous nephropathy (MN) [ 4 ]. Hypokalemia is the most common electrolyte abnormality in patients with RTA.…”

Section: Discussionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

“…This causes an increase in membrane permeability and the release of intracellular creatine kinase (CK) into the blood [ 6 ]. Hypokalemic paralysis as a presenting manifestation of SS is extremely rare [ 4 , 5 , 6 ].…”

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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“…Since we did not find sufficient evidence in this patient to support a diagnosis of other autoimmune diseases such as systemic lupus erythematosus, she was ultimately diagnosed with pSS according to guidelines for the management of SS [ 10 – 12 , 19 , 24 ]. In addition to the typical clinical manifestations of dry mouth and eye with the function of salivary and lacrimal glands impaired, approximately 50% of patients with SS present multi-system damage due to the infiltration of lymphocytes in the lung, liver, kidney, skin, blood vessels, and nervous system [ 10 , 12 , 14 ]. The infiltration of ductal and perivascular lymphocytes in salivary and lacrimal glands is the main histological feature of SS.…”

Section: Discussionmentioning

confidence: 99%

“…The incidence of this disease is approximately 12.3/1000, and approximately 2~5% of patients have complications of lymphoma [ 10 – 13 ]. It is extremely rare to report SS with renal tubular acidosis (RTA) and hypokalemia, especially with acute myopathy as the initial clinical manifestation, although approximately 30% of SS patients are associated with renal interstitial injury [ 11 , 12 , 14 – 16 ].…”

Section: Introductionmentioning

confidence: 99%

Severe Hypokalemia Complicated by Acute Myopathy: Initial Manifestation of Primary Sjögren’s Syndrome-Associated Renal Tubular Acidosis

Gao,

Milebe Nkoua,

Chai

2023

Am J Case Rep

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Patient: Female, 35-year-old Final Diagnosis: Primary Sjögren’s syndrome • primary Sjögren’s syndrome-associated renal tubular acidosis Symptoms: Pain • weakness Clinical Procedure: — Specialty: Critical Care Medicine Objective: Rare coexistence of disease or pathology Background: Severe hypokalemia, which often causes life-threatening malignant arrhythmias, is usually first diagnosed in the Emergency Department (ED). It is important to note that hypokalemia is often closely and complexly related to renal tubular acidosis (RTA) associated with autoimmune diseases such as Sjögren’s syndrome (SS), especially in females with acute myopathy or acute liver injury (ALI). Severe hypokalemia can directly cause muscle injury, which can lead to hyper-creatine kinaseemia (HCK) and ALI, while SS can also directly cause hypokalemia, HCK, and even ALI and renal tubular/interstitial injury. Therefore, by reporting a rare case of SS-associated RTA (SS-RTA), we systematically reviewed the relationship between SS-RTA and severe hypokalemia, which may be beneficial to increase attention on this topic. Case Report: A 35-year-old female patient who presented to the ED primarily for limb weakness symptoms was initially diagnosed with severe hypokalemia, acute myopathy, and ALI. She was eventually diagnosed with primary SS (pSS) and SS-RTA, although she did not present with the typical dry mouth, dry eyes, and other clinical manifestations of SS. Conclusions: Severe hypokalemia is a serious life-threatening emergency, and although the differential diagnosis is very broad, we should be aware of RTA associated with autoimmune diseases such as SS in female patients, especially when combined with clinical manifestations such as acute myopathy and ALI that cannot be explained by other causes. Simultaneously, we hope to be able to guide emergency physicians encountering similar patients to complete the diagnostic and therapeutic process.

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“…Renal tubular dysfunction due to interstitial nephritis is considered the underlying pathophysiology connecting dRTA and pSS [ 5 , 7 ]; however, the mechanism of RTA is not yet fully understood. Several kidney specific proteins are associated with renal tubular dysfunction [ 8 , 9 ], whereas one of the proposed hypotheses of RTA is that dysfunction of the vacuolar-type H + -ATPases (V-ATPases) is responsible for the defect in urinary acidification leading to metabolic acidosis [ 10 ]. The aim of this study was to highlight the clinical course of renal manifestations in patient with pSS and to investigate the potential association between RTA and V-ATPases in pSS.…”

Section: Introductionmentioning

confidence: 99%

Renal tubular acidosis without interstitial nephritis in Sjögren’s syndrome: a case report and review of the literature

et al. 2023

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Background Renal tubular acidosis is the principal clinical feature associated with tubulointerstitial nephritis in patients with primary Sjögren’s syndrome. Renal tubular dysfunction due to interstitial nephritis has been considered the underlying pathophysiology connecting renal tubular acidosis and primary Sjögren’s syndrome. However, the detailed mechanisms underlying the pathophysiology of renal tubular acidosis in primary Sjögren’s syndrome is not fully understood. Case presentation A 30-year-old woman was admitted with complaints of weakness in the extremities. The patient was hospitalized thirteen years earlier for similar issues and was diagnosed with hypokalemic paralysis due to distal renal tubular acidosis with primary Sjögren’s syndrome. This diagnosis was based on a positive Schirmer's test. Besides, anti-Sjögren’s syndrome-related antigen A was also detected. Laboratory tests indicated distal RTA; however, a renal biopsy showed no obvious interstitial nephritis. Laboratory tests conducted during the second admission indicated distal renal tubular acidosis. Therefore, a renal biopsy was performed again, which revealed interstitial nephritis. Histological analysis of acid–base transporters revealed the absence of vacuolar type H+-ATPases in the collecting duct. The vacuolar type H+-ATPase was also absent in the past renal biopsy, suggesting that the alteration in acid–base transporters is independent of interstitial nephritis. Conclusions This case study demonstrates that vacuolar-type H+-ATPases are associated with distal renal tubular acidosis, and distal renal tubular acidosis precedes interstitial nephritis in patients with primary Sjögren’s syndrome.

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Recurrent Attacks of Hypokalemic Quadriparesis: An Unusual Presentation of Primary Sjögren Syndrome

Cited by 6 publications

References 19 publications

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

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

Severe Hypokalemia Complicated by Acute Myopathy: Initial Manifestation of Primary Sjögren’s Syndrome-Associated Renal Tubular Acidosis

Renal tubular acidosis without interstitial nephritis in Sjögren’s syndrome: a case report and review of the literature

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