Hypokalemia Related to Distal Renal Tubular Acidosis as an Initial Presentation of Primary Sjogren’s Syndrome

I Gede Yasa Asmara, Maruhum Bonar Hasiholan Marbun

Abstract


Hypokalemia due to loss of potassium through the kidneys can be caused by distal Renal Tubular Acidosis (dRTA). The etiology of dRTA can be primary due to genetic defects or secondary to autoimmune diseases, especially Sjogren’s syndrome (SS). The occurrence of dRTA in SS patients is low, at only 5% of cases. This case was interesting because dRTA was the initial clinical manifestation that led to the diagnosis of SS in the patient. A 48-year-old woman came with complaints of recurrent weakness. The patient was routinely hospitalized with severe hypokalemia and received potassium supplementation. The diagnosis of dRTA was based on repeated weakness, normal blood pressure, severe and recurrent hypokalemia, high urinary potassium, alkaline urine, low plasma bicarbonate, and standard anion gap metabolic acidosis. The diagnosis of SS in this patient was confirmed based on dry eyes, dry mouth, positive Schirmer’s test, and positive autoantibodies to SS-A and Ro-52. There was a delay in the diagnosis of SS for two years in this patient because the complaints were initially subtle and non-specific. The hypokalemia in this patient was secondary to dRTA associated with primary SS. The possibility of an underlying autoimmune disorder should be considered in a patient presenting with recurrent severe hypokalemia. dRTA, as the etiology of hypokalemia, can be a gateway to the diagnosis of SS. In this patient, complaints related to dRTA appeared before the onset of sicca symptoms, and the diagnosis of SS was established.


Keywords


Distal Renal Tubular Acidosis; Hypokalemia; Sjogren's Syndrome

References


Patel JK. Distal renal tubular acidosis due to primary Sjögren’s syndrome: Presents as hypoakalemic paralysis with hypokalemia-induced nephrogenic diabetes insipidus. Saudi J Kidney Dis Transplant. 2021;32(3):851-4.

Giglio S, Montini G, Trepiccione F, Gambaro G, Emma F. Distal renal tubular acidosis: a systematic approach from diagnosis to treatment. J Nephrol. 2021;34(6):2073-83.

Wang Q, Dai SM. Premature onset of Sjögren’s syndrome is prone to be complicated with renal tubular acidosis. Int J Rheum Dis. 2020;23(10):1421-5.

Ho K, Dokouhaki P, McIsaac M, Prasad B. Renal tubular acidosis as the initial presentation of Sjögren’s syndrome. BMJ Case Rep. 2019;12(8):10-3.

Chalkia A, Giannou1 P, Thomas K, Vassilopoulos D, Petras D. Distal renal tubular acidosis and nephrocalcinosis as initial manifestation of primary sjögren’s syndrome. Saudi J Kidney Dis Transplant. 2021;32(5):1471-4.

Kardalas E, Paschou SA, Anagnostis P, Muscogiuri G, Siasos G, Vryonidou A. Hypokalemia: A clinical update. Endocr Connect. 2018;7(4):R135-R146.

Zhou Y, Huang N, Cao G, Luo Y. Type I renal tubular acidosis caused by Sjögren’s syndrome with hypokalemia as the first symptom: a case report. J Int Med Res. 2019;48(2):0300060519880765.

Trepiccione F, Walsh SB, Ariceta G, Boyer O, Emma F, Camilla R, et al. Distal renal tubular acidosis: ERKNet/ESPN clinical practice points. Nephrol Dial Transplant. 2021;36(9):1585-96.

Louis-Jean S, Ching PR, Wallingford A. Distal Renal Tubular Acidosis in Sjögren’s Syndrome: A Case Report. Cureus. 2020;12(10):e10962.

Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjögren’s syndrome: A revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002;61(6):554-8.

Aiyegbusi O, McGregor L, McGeoch L, Kipgen D, Geddes CC, Stevens KI. Renal disease in primary Sjögren’s syndrome. Rheumatol Ther. 2021;8(1):63-80.

Toosi TD, Naderi N, Movassaghi S, Seradj MH, Khalvat A, Shahbazi F. Secondary Sjogren’s syndrome presenting with hypokalemic periodic paralysis. Oxford Med Case Reports. 2014;2014(8):135-7.

Ramos-Casals M, Brito-Zerón P, Bombardieri S, et al. EULAR recommendations for the management of Sjögren’s syndrome with topical and systemic therapies. Ann Rheum Dis. 2020;79(1):3-18.

Narayan R, Abdulla MC, Alungal J, Krishnadas NC. Kidney diseases and transplantation case report distal renal tubular acidosis in Sjögren’s syndrome. Saudi J Kidney Dis Transplant. 2018;29(2):470-3.


Full Text: PDF

Refbacks

  • There are currently no refbacks.


Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.