Case Report

Unusual Cause of Hypokalemic Paralysis in Aged Men: Sjögren Syndrome

Authors: Chih-Jen Cheng, MD, Jainn-Shiun Chiu, MD, Chun-Chi Chen, MD, Shih-Hua Lin, MD

Abstract

Hypokalemic paralysis is a less recognized but reversible disorder in elderly patients. This report describes two elderly Chinese males (age 74 and 78 years) who had progressive muscle weakness and eventually paralysis. Physical examination showed symmetrical flaccid paralysis of extremities. Both had the major biochemical abnormality of profound hypokalemia (1.4 and 1.8 mmol/L) accompanied by high urine K+ excretion and hyperchloremic metabolic acidosis. A positive urine anion gap and alkaline urine pointed to the diagnosis of distal renal tubular acidosis. Large doses of potassium chloride supplementation were required to restore muscle strength. Pertinent investigations, including elevated titers of antinuclear antibody and rheumatoid factor, positive anti-Ro antibody, low serum C3 and C4 levels, and delayed saliva excretion on salivary scintigraphy suggested Sjögren syndrome. Despite the lack of sicca syndrome at the initial presentation, both had development of typical sicca syndrome and positive Schirmer test at the 5-month and 1-year follow-up, respectively. Potassium citrate supplement and prednisolone therapy completely corrected the hypokalemia and metabolic acidosis. Extraglandular involvement with distal renal tubular acidosis preceding the typical sicca syndrome may induce hypokalemic paralysis and unveil Sjögren syndrome in elderly males.


Key Points


* Hypokalemic paralysis is a less recognized but potentially reversible electrolyte disorder seen in the elderly.


* Hypokalemic paralysis caused by distal renal tubular acidosis may unveil Sjögren syndrome in elderly patients, even without initial typical sicca syndrome.


* Although Sjögren syndrome usually affects middle-aged females, it can affect elderly males.


* Distal renal tubular acidosis, a marker of more severe renal (extraglandular) involvement, can predate the typical exocrine involvement in Sjögren syndrome.


* The optimal treatment for Sjögren syndrome associated with distal renal tubular acidosis-related hypokalemic paralysis not only corrects the acidosis and hypokalemia but also controls the underlying renal involvement.

This content is limited to qualifying members.

Existing members, please login first

If you have an existing account please login now to access this article or view purchase options.

Purchase only this article ($25)

Create a free account, then purchase this article to download or access it online for 24 hours.

Purchase an SMJ online subscription ($75)

Create a free account, then purchase a subscription to get complete access to all articles for a full year.

Purchase a membership plan (fees vary)

Premium members can access all articles plus recieve many more benefits. View all membership plans and benefit packages.

References

1. Lin SH, Davids MR, Halperin ML. Hypokalaemia and paralysis. QJM 2003;96:161–169.
 
2. Pokorny G, Sonkodi S, Ivanyi B, et al. Renal involvement in patients with primary Sjogren's syndrome. Scand J Rheumatol 1989;18:231–234.
 
3. al-Jubouri MA, Jones S, Macmillan R, et al. Hypokalaemic paralysis revealing Sjogren's syndrome in an elderly man. J Clin Pathol 1999;52:157–158.
 
4. Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjogren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis2002;61:554–558.
 
5. Lin SH, Lin YF, Chen DT, et al. Laboratory tests to determine the cause of hypokalemia and paralysis. Arch Intern Med 2004;164:1561–1566.
 
6. Pun KK, Wong CK, Tsui EY, et al. Hypokalemic periodic paralysis due to the Sjögren syndrome in Chinese patients. Ann Intern Med 1989;110:405–406.
 
7. Shioji R, Furuyama T, Onodera S, et al. Sjogren's syndrome and renal tubular acidosis. Am J Med1970;48:456–463.
 
8. Eriksson P, Denneberg T, Enestrom S, et al. Urolithiasis and distal renal tubular acidosis preceding primary Sjogren's syndrome: a retrospective study 5–53 years after the presentation of urolithiasis. J Intern Med 1996;239:483–488.
 
9. Pertovaara M, Korpela M, Kouri T, et al. The occurrence of renal involvement in primary Sjogren's syndrome: a study of 78 patients. Rheumatology 1999;38:1113–1120.
 
10. Siamopoulos KC, Mavridis AK, Elisaf M, et al. Kidney involvement in primary Sjogren's syndrome.Scand J Rheumatol 1986;61:156–160.
 
11. Konishi K, Hayashi M, Saruta T. Renal tubular acidosis with autoantibody directed to renal collecting-duct cells. N Engl J Med 1994;331:1593–1594.
 
12. DeFranco PE, Haragsim L, Schwartz PG, et al. Absence of vacuolar H+-ATPase pump in the collecting duct of a patient with hypokalemic distal renal tubular acidosis and Sjogren's syndrome. J Am Soc Nephrol 1995;6:295–301.
 
13. Lin SH, Cheema-Dhadli S, Gowrishankar M, et al. Control of excretion of potassium lessons from studies during prolonged total fasting in human subjects. Am J Physiol 1997;273:F796–F800.
 
14. Shintani S, Shiigai T, Tsukagoshi H. Marked hypokalemic rhabdomyolysis with myoglobinuria due to diuretic treatment. Eur Neurol 1991;31:396–398.
 
15. Cheng CJ, Chen YH, Chau T, et al. A hidden cause of hypokalemic paralysis in a patient with prostate cancer. Support Care Cancer 2004;12:810–812.