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Year : 2020  |  Volume : 11  |  Issue : 1  |  Page : 51-53

Hypokalemic paralysis due to primary sjögren syndrome: Literature review

Department of Neurology; Department of Medicine, Federal University of Santa Maria, Santa Maria, Brazil

Date of Submission17-Oct-2019
Date of Acceptance26-Dec-2020
Date of Web Publication12-Feb-2020

Correspondence Address:
Mr. Jamir Pitton Rissardo
Rua Roraima, Santa Maria, Rio Grande do Sul
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/INJMS.INJMS_135_19

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How to cite this article:
Rissardo JP, Caprara AL. Hypokalemic paralysis due to primary sjögren syndrome: Literature review. Indian J Med Spec 2020;11:51-3

How to cite this URL:
Rissardo JP, Caprara AL. Hypokalemic paralysis due to primary sjögren syndrome: Literature review. Indian J Med Spec [serial online] 2020 [cited 2023 Jun 9];11:51-3. Available from: http://www.ijms.in/text.asp?2020/11/1/51/278088

Dear Editor,

We read an article in “Indian Journal of Medical Specialities” with great interest. Singh et al., in 2019, reported a case of an adult female who presented with acute quadriplegia and respiratory distress. A diagnosis of renal tubular acidosis (RTA) type 1 (distal) was made. On further tests, antiSjögren's syndrome (SS)-A/Ro and antiSS-B/La were strongly positive. A diagnosis of SS with hypokalemia due to RTA and demyelinating pure motor axonal polyneuropathy was made. After 3 months, the patient returned with complaints of progressive weakness of limbs and breathing difficulty. Thus, she was diagnosed with Guillain–Barré syndrome.[1]

Hypokalemic paralysis due to primary SS was rarely reported in literature. We identified 59 individuals, and we added more one unreported case from our institution [Table 1]. A literature search was performed in Embase, Google Scholar, Lilacs, Medline, Scielo, and ScienceDirect, on a set of terms that included hypokalemic paralysis, SS, and RTA. We included only articles in English or Spanish with a complete report of the treatment.[1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20],[21],[22],[23],[24],[25],[26],[27],[28],[29],[30],[31],[32]
Table 1: Reports of patients with hypokalemic paralysis due to primary Sjögren syndrome

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We would like to address some important topics extracted from [Table 1]. First, the majority of the individuals were from Asian countries, which accounted for at least one in every three individuals reported. This could be explained by a high prevalence of SS in these countries, even though the rates of autoimmune diseases be the same between the American and Asian populations.[6] Second, it is observed a female prevalence in the studies with this severe complication of SS. In the reports with male, nephrocalcinosis was more frequent, but worse follow-up was markedly noted with females.[9]

Three possible pathophysiological mechanisms were already described to explain the occurrence of hypokalemic paralysis due to primary SS. In this context, an assumption based on antibodies to H+-ATPase and carbonic anhydrase leading to a urine pH >5.5 and a positive urinary anion gap was proposed.[15] The second hypothesis could be a defective kinase related to a component of the regulator of the ATPase.[24] However, these were only observed in a portion of the reported cases.[21] Another possible explanation is the antibodies anti-SSA/Ro, leading to a direct distal RTA.[9]

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