|LETTER TO THE EDITOR
|Year : 2020 | Volume
| Issue : 1 | Page : 51-53
Hypokalemic paralysis due to primary sjögren syndrome: Literature review
Jamir Pitton Rissardo, Ana Letícia Fornari Caprara
Department of Neurology; Department of Medicine, Federal University of Santa Maria, Santa Maria, Brazil
|Date of Submission||17-Oct-2019|
|Date of Acceptance||26-Dec-2020|
|Date of Web Publication||12-Feb-2020|
Mr. Jamir Pitton Rissardo
Rua Roraima, Santa Maria, Rio Grande do Sul
Source of Support: None, Conflict of Interest: None
|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 2020 May 26];11:51-3. Available from: http://www.ijms.in/text.asp?2020/11/1/51/278088
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.
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.,,,,,,,,,,,,,,,,,,,,,,,,,,,,,,,
|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. 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.
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. The second hypothesis could be a defective kinase related to a component of the regulator of the ATPase. However, these were only observed in a portion of the reported cases. Another possible explanation is the antibodies anti-SSA/Ro, leading to a direct distal RTA.
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