|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 Sep 24];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|
Click here to view
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.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Singh VP, Khullar J, Garg A, Vardani A. Acute quadriplegia: An interesting case with etiological and pathophysiological disparity. Indian J Med Spec 2019;10:162. [Full text]
Carminati G, Chena A, Orlando JM, Russo S, Salomón S, Carena JA. Distal renal tubular acidosis with rhabdomyolysis as the presenting form in 4 pregnant women. Nefrologia 2001;21:204-8.
Chang YC, Huang CC, Chiou YY, Yu CY. Renal tubular acidosis complicated with hypokalemic periodic paralysis. Pediatr Neurol 1995;13:52-4.
Cheng CJ, Chiu JS, Chen CC, Lin SH. Unusual cause of hypokalemic paralysis in aged men: Sjögren syndrome. South Med J 2005;98:1212-5.
Comer DM, Droogan AG, Young IS, Maxwell AP. Hypokalaemic paralysis precipitated by distal renal tubular acidosis secondary to Sjögren's syndrome. Ann Clin Biochem 2008;45:221-5.
Dasari S, Naha K, Vivek G, Acharya V, Hande M. Primary presentation with acute flaccid quadriparesis in Sjogren's syndrome sans sicca. BMJ Case Rep 2013;2013. pii: bcr2012008172.
Eriksson P, Denneberg T, Eneström S, Johansson B, Lindström F, Skogh T. Urolithiasis and distal renal tubular acidosis preceding primary Sjögren's syndrome: A retrospective study 5-53 years after the presentation of urolithiasis. J Intern Med 1996;239:483-8.
Fujimoto T, Shiiki H, Takahi Y, Dohi K. Primary Sjögren's syndrome presenting as hypokalaemic periodic paralysis and respiratory arrest. Clin Rheumatol 2001;20:365-8.
Garza-Alpirez A, Arana-Guajardo AC, Esquivel-Valerio JA, Villarreal-Alarcón MA, Galarza-Delgado DA. Hypokalemic paralysis due to primary Sjögren syndrome: Case report and review of the literature. Case Rep Rheumatol 2017;2017:7509238.
Goroshi M, Khare S, Jamale T, Shah NS. Primary Sjogren's syndrome presenting as hypokalemic paralysis: A case series. J Postgrad Med 2017;63:128-31.
] [Full text]
Kawashima M, Amano T, Morita Y, Yamamura M, Makino H. Hypokalemic paralysis and osteomalacia secondary to renal tubular acidosis in a case with primary Sjögren's syndrome. Mod Rheumatol 2006;16:48-51.
Khandelwal D, Bhattacharya S, Khadgawat R, Kaur S, Tandon N, Ammini AC. Hypokalemic paralysis as a presenting manifestation of primary Sjögren's syndrome: A report of two cases. Indian J Endocrinol Metab 2012;16:853-5.
Liao CY, Wang CC, Chen IH, Shiang JC, Liu MY, Tsai MK. Hypokalemic paralysis as a presenting manifestation of primary Sjögren's syndrome accompanied by vitamin D deficiency. Intern Med 2013;52:2351-3.
Logan JL, Ahmed J. Critical hypokalemic renal tubular acidosis due to Sjögren's syndrome: Association with the purported immune stimulant echinacea. Clin Rheumatol 2003;22:158-9.
Mugundhan K, Vasif MM, Nidhin PD, Prakash G, Balamurugan N, Sivakumar KG, et al.
Hypokalemic paralysis in Sjogren's syndrome secondary to renal tubular acidosis. Journal of Association of Physicians of India. 2016;64:72.
Muthukrishnan J, Dawra S, Marwaha V, Narayanan CS. Sjögren's syndrome presenting as hypokalemic paralysis. Med J Armed Forces India 2015;71:S172-4.
Naik M, Bhat T, Naqash M, Qadri M, Yusuf I, Ali I, et al
. Hypokalemic quadriparesis in an elderly female. Indian J Nephrol 2012;22:402-3.
] [Full text]
Paliwal VK, Rai AS, Kumar S, Verma R, Agarwal V. Proximal muscle weakness with overlying hypokalemic periodic paralysis in Sjögren syndrome: Report of 6 cases. J Clin Rheumatol 2020;26:24-7.
Palkar AV, Pillai S, Rajadhyaksha GC. Hypokalemic quadriparesis in Sjogren syndrome. Indian J Nephrol 2011;21:191-3.
] [Full text]
Prakash EB, Fernando ME, Sathiyasekaran M, Bhoopathy RM, Jayanth JJ, Samuel J. Primary Sjögren's syndrome presenting with distal, renal tubular acidosis and rhabdomyolysis. J Assoc Physicians India 2006;54:949-50.
Rajagopala S, Danigeti G, Subrahmanyan D. An unusually dry story. Indian J Crit Care Med 2015;19:550-3.
] [Full text]
Rao N, John M, Thomas N, Rajaratnam S, Seshadri MS. Aetiological, clinical and metabolic profile of hypokalaemic periodic paralysis in adults: A single-centre experience. Natl Med J India 2006;19:246-9.
Sarah S, Lijo G, Sukanya E, Rajasekaran D. Renal tubular acidosis due to Sjogren's syndrome presenting as hypokalemic quadriparesis: A report of two cases. Indian J Nephrol 2015;25:386-7.
] [Full text]
Seirafian S, Shafie M, Abedini A, Pakzad B, Roomizadeh P. Recurrent attacks of hypokalemic quadriparesis: An unusual presentation of primary Sjögren syndrome. Intern Med 2016;55:1797-800.
Sengul E, Bunul F, Yazici A, Sengul A, Dindar S, Halhalli GS, et al
. An unusual initial presentation of Sjögren's syndrome: Severe hypokalemic paralysis secondary to distal renal tubular acidosis. Eurasian J Med 2013;45:218-21.
Singhvi JP, Ganguli A, Kaur B. Primary Sjogren's syndrome presenting as acute flaccid quadriplegia. Ann Neurosci 2010;17:98-100.
Skalova S, Minxova L, Slezak R. Hypokalaemic paralysis revealing Sjogren's syndrome in a 16-year old girl. Ghana Med J 2008;42:124-8.
Soy M, Pamuk ON, Gerenli M, Celik Y. A primary Sjögren's syndrome patient with distal renal tubular acidosis, who presented with symptoms of hypokalemic periodic paralysis: Report of a case study and review of the literature. Rheumatol Int 2005;26:86-9.
Taylor I, Parsons M. Hypokalemic paralysis revealing Sjögren's syndrome. J Clin Neurosci 2004;11:319-21.
Vaidya G, Ganeshpure S. Sjogren's syndrome with distal renal tubular acidosis presenting as hypokalaemic paralysis. BMJ Case Rep 2012;2012. pii: bcr2012007568.
Vasquez-Rios G, Westrich DJ Jr, Philip I, Edwards JC, Shieh S. Distal renal tubular acidosis and severe hypokalemia: A case report and review of the literature. J Med Case Rep 2019;13:103.
Yılmaz H, Kaya M, Özbek M, ÜUreten K, Safa Yıldırım İ. Hypokalemic periodic paralysis in Sjogren's syndrome secondary to distal renal tubular acidosis. Rheumatol Int 2013;33:1879-82.