Hypokalemic paralysis as primary presentation of Sjögren’s syndrome – A clinical case report
DOI:
https://doi.org/10.51168/sjhrafrica.v5i12.1486Keywords:
Sjogren syndrome, hypokalemia, hypokalemic paralysis, renal tubular acidosisAbstract
Sjogren’s syndrome is an autoimmune rheumatic disease characterized by lymphocytic infiltration of exocrine glands resulting in multi system presentations including xerostomia and dry eyes. A 47-year-old woman presented with generalized weakness, difficulty in walking, nausea and pain abdomen. She had reduced muscle power (2/5) with reduced tone, and absent deep tendon reflexes in all four limbs. Further investigation revealed severe hypokalemia with metabolic acidosis. She was treated with intravenous potassium chloride and diagnosed with Sjogren's syndrome based on her serology profile. After treatment, her condition improved. Hypokalemia occurs due to increased renal excretions of potassium. Distal Renal Tubular Acidosis is a major cause of potassium loss in urine. Sjogren's Syndrome is an important cause of Distal Renal Tubular Acidosis, which can present as severe hypokalemia. Although it commonly presents with symptoms like dry eyes and dry mouth, it can rarely present as Distal Renal Tubular Acidosis and hypokalemia as the primary presentation. Sjogren's syndrome should be suspected in patients with hypokalemia with features of Distal Renal Tubular Acidosis.
References
Brito-Zerón P, Baldini C, Bootsma H, Bowman SJ, Jonsson R, Mariette X, Sivils K, Theander E, Tzioufas A, Ramos-Casals M (7 July 2016). "Sjögren syndrome". Nature Reviews Disease Primers. 2 (1): 16047. doi:10.1038/nrdp.2016.47
Aiyegbusi, O., McGregor, L., McGeoch, L. et al. Renal Disease in Primary Sjögren’s Syndrome. Rheumatol Ther 8, 63–80 (2021). https://doi.org/10.1007/s40744-020-00264-x
Siddamreddy S, Dandu VH. Thyrotoxic Periodic Paralysis. [Updated 2023 Jul 24]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK560670/
Jung, S. W., Park, E. J., Kim, J. S., Lee, T. W., Ihm, C. G., Lee, S. H., Moon, J. Y., Kim, Y. G., & Jeong, K. H. (2017). Renal Tubular Acidosis in Patients with Primary Sjögren's Syndrome. Electrolyte & blood pressure : E & BP, 15(1), 17–22. https://doi.org/10.5049/EBP.2017.15.1.17
Giglio, S., Montini, G., Trepiccione, F. et al. Distal renal tubular acidosis: a systematic approach from diagnosis to treatment. J Nephrol 34, 2073–2083 (2021). https://doi.org/10.1007/s40620-021-01032-y
Poux JM, Peyronnet P, Le Meur Y, Favereau JP, Charmes JP, Leroux-Robert C. Hypokalemic quadriplegia and respiratory arrest revealing primary Sjögren’s syndrome. Clin Nephrol. 1992;37(4):189-191.
HYPOKALEMIA. In: Kasper DLD, Fauci ASA, Hauser SLS, et al, eds. Harrison's Manual of Medicine. McGraw Hill Inc.; 2020. https://harrisons.unboundmedicine.com/harrisons/view/Harrisons-Manual-of-Medicine/623468/all/HYPOKALEMIA. Accessed December 26, 2024.
Sedhain A, Acharya K, Sharma A, Khan A, Adhikari S. Renal tubular acidosis and hypokalemic paralysis as a first presentation of primary Sjögren’s syndrome. Case Reports in Nephrology. 2018 Oct 16;2018:1–4. https://doi.org/10.1155/2018/9847826
Agha RA, Franchi T, Sohrabi C, Mathew G, Kerwan A, Thoma A, et al. The SCARE 2020 Guideline: Updating Consensus Surgical CASE Report (SCARE) guidelines. International Journal of Surgery. 2020 Dec 1;84:226–30. https://doi.org/10.1016/j.ijsu.2020.10.034
Kardalas E, Paschou SA, Anagnostis P, Muscogiuri G, Siasos G, Vryonidou A. Hypokalemia: a clinical update. Endocrine Connections. 2018 Apr 1;7(4):R135–46. https://doi.org/10.1530/EC-18-0109
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. Journal of International Medical Research. 2020;48(2). Doi:10.1177/0300060519880765
Mortazavi, H., Baharvand, M., Movahhedian, A., Mohammadi, M., & Khodadoustan, A. (2014). Xerostomia due to systemic disease: a review of 20 conditions and mechanisms. Annals of medical and health sciences research, 4(4), 503–510. https://doi.org/10.4103/2141-9248.139284
Duan, N., Li, Z., Fan, Y. et al. Related factors of renal injury in primary Sjögren’s syndrome. Immun Ageing 20, 48 (2023). https://doi.org/10.1186/s12979-023-00375-3
Bagga A, Sinha A. Evaluation of renal tubular acidosis. Indian J Pediatr. 2007 Jul;74(7):679-86. doi: 10.1007/s12098-007-0120-0. PMID: 17699978.
Sengul E, Bunul F, Yazici A, et al. An Unusual Initial Presentation of Sjögren’s Syndrome: Severe Hypokalemic Paralysis Secondary to Distal Renal Tubular Acidosis. Eurasian J Med. 2013;45(3):218-221. Doi:10.5152/eajm.2013.43
Krishna Bahadur Sodari, Pragyan Basnet, Krishna Adhikari. Sjogren’s Syndrome in the Mask of Hypokalemia: A Case Report. J Nepal Med Assoc 2024;62(280):841-3. Doi:10.31729/jnma.8836
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