Hypokalemic paralysis as an initial presentation of Sjogren syndrome

Meena, Durga Shankar; Kumar, Deepak; Bohra, Gopal Krishana; Bhambu, Sunil Kumar

doi:10.4103/aam.aam_34_19

Cited by 6 publications

(4 citation statements)

References 9 publications

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“… 2 Distal renal tubular acidosis (dRTA) occurs in about 25% of patients with pSS but only 8% of patients with distal renal tubular acidosis also have a prior diagnosis of pSS. 5 , 6 Usually, dRTA presents with mild hypokalemia and normal anion gap acidosis. However, very few cases reporting severe hypokalemia in dRTA leading to muscle paralysis have been published in the literature.…”

Section: Discussionmentioning

confidence: 99%

Hypokalemic Paralysis Revealing Primary Sjogren's Syndrome: A Case Report

Khanal,

Paudel,

Chapagain

et al. 2023

J Nepal Med Assoc

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Sjogren's syndrome is a rare chronic autoimmune disease characterised by dry eyes and dry mouth due to autoimmune destruction of the lacrimal and salivary glands, which can occur concurrently with other autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, or thyroiditis. It can lead to renal complications such as interstitial nephritis and glomerulonephritis, with distal/ type 1 renal tubular acidosis which may result in life-threatening electrolyte imbalance. We present a case of a 35-year-old female who presented with complaints of multiple episodes of muscle weakness. Type 1 renal tubular acidosis was discovered to be the cause of her symptoms which lead to the subsequent diagnosis of Sjogren’s syndrome. This is rare presentation of Sjogren’s syndrome, and it poses a challenge to diagnosis. Early detection and diagnosis of Sjogren's syndrome might be difficult due to existing diagnostic criteria, which contributes to a higher likelihood of missed diagnosis.

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Section: Discussionmentioning

confidence: 99%

Hypokalemic Paralysis Revealing Primary Sjogren's Syndrome: A Case Report

Khanal,

Paudel,

Chapagain

et al. 2023

J Nepal Med Assoc

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“…Renal involvement is common in SS, usually in the form of tubulointerstitial nephritis. Most cases, however, are either mild or asymptomatic [ 6 ]. dRTA with severe hypokalemia leading to quadriparesis, although rare, has been reported in several case reports as an initial presentation of SS [ 7 - 11 ].…”

Section: Discussionmentioning

confidence: 99%

Hypokalemic Quadriparesis As Initial Presentation of Secondary Sjogren Syndrome With Associated Autoimmune Thyroiditis: A Case Report

Fazal¹,

Rehman²,

Tahir³

et al. 2022

Cureus

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Sjögren's syndrome is an autoimmune disorder typically presenting as dry mouth and eyes (sicca syndrome). However, the disease can involve any organ, complicating diagnosis. Renal involvement may manifest as distal renal tubular acidosis, leading to hypokalemia. We report a case of a 25-year-old woman presenting with progressive quadriparesis and vomiting. She had severe hypokalemic paralysis due to distal renal tubular acidosis. The patient was diagnosed with secondary Sjögren syndrome with autoimmune thyroiditis. She recovered completely with potassium supplementation.

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“…Two additional case reports with this association were published in 2020, and another study published recently showed a prevalence of 14.81% of DRTA (16 patients) in a cohort of 108 patients with SLE [ 6 , 7 , 8 ]. Generally, the majority of cases with complete DRTA published in literature are diagnosed due to severe symptoms associated with hypokalemia, such as quadriparesis or even respiratory arrest, and therefore, the real prevalence of DRTA associated with autoimmune diseases is difficult to estimate [ 6 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 ]. Therefore, urinary acidification tests are essential in diagnosing those patients who did not develop any symptoms and would otherwise escape undiagnosed.…”

Section: Epidemiology Clinical Presentation and Diagnosismentioning

confidence: 99%

Distal Renal Tubular Acidosis in Patients with Autoimmune Diseases—An Update on Pathogenesis, Clinical Presentation and Therapeutic Strategies

Ungureanu

Ismail

2022

Biomedicines

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Distal renal tubular acidosis (DRTA) has been reported in association with autoimmune diseases, such as Sjögren’s syndrome, systemic lupus erythematosus (SLE), autoimmune hepatitis, primary biliary cirrhosis, rheumatoid arthritis and autoimmune thyroiditis. Whether we talk about the complete or incomplete form of DRTA associated with autoimmune diseases, the real incidence is unknown because asymptomatic patients usually are not identified, and most of the reported cases are diagnosed due to severe symptoms secondary to hypokalemia, a frequent finding in these cases. The mechanisms involved in DRTA in patients with autoimmune diseases are far from being fully elucidated and most of the data has come from patients with Sjögren’s syndrome. This review will present different hypotheses raised to explain this association. Also, aiming for a better understanding of the association between autoimmune diseases and DRTA, our review summarizes data from 37 case reports published in the last five years. We will emphasize data regarding clinical presentation, biological alterations, treatment and outcome. A very important question is whether immunosuppressive therapy is helpful in DRTA associated with autoimmune diseases. Because the pathology is rather rare, treatment is not standardized, and reported results are often contradictory. Corticosteroids are frequently used, but multiple other immunosuppressive drugs have been proposed and will be approached in this review.

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Hypokalemic paralysis as an initial presentation of Sjogren syndrome

Cited by 6 publications

References 9 publications

Hypokalemic Paralysis Revealing Primary Sjogren's Syndrome: A Case Report

Hypokalemic Paralysis Revealing Primary Sjogren's Syndrome: A Case Report

Hypokalemic Quadriparesis As Initial Presentation of Secondary Sjogren Syndrome With Associated Autoimmune Thyroiditis: A Case Report

Distal Renal Tubular Acidosis in Patients with Autoimmune Diseases—An Update on Pathogenesis, Clinical Presentation and Therapeutic Strategies

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