Abstract
A 14-year-old female came with the history of sudden onset weakness; during work up, she was found to have hyperchloremic metabolic acidosis with normal anion gap and normal renal function suggesting the possibility of renal tubular acidosis (RTA). On further evaluation of RTA, she had positive antinuclear antibody, anti-Ro, and anti-La antibodies. On nuclear scan of salivary glands, her left parotid gland was nonfunctional. Her parotid biopsy revealed dilated interlobular ducts engulfed by lymphoid cells. She also had autoimmune hypothyroidism as suggested by raised TSH and positive anti-TPO antibodies. At admission, her serum potassium levels were low and she was treated with intravenous potassium chloride. After she recovered from acute hypokalemic paralysis, she was started on oral potassium citrate along with phosphate supplements, hydroxychloroquine, oral prednisolone and thyroxine supplements. Over the next 6 months, she has significant reduction in the dosage of potassium, bicarbonate and phosphate and gained 3 kg of weight and 3.5 cm of height. As primary Sjogren syndrome itself is rare in pediatric population and its association with renal tubular acidosis is even rarer, we suggest considering Sjogren syndrome as a differential diagnosis during the RTA work-up is worth trying.
References
Vissink A, Bootsma H, Spijkervet F, et al. Current and future challenges in primary Sjogren’s syndrome. Curr Pharm Biotechnol. 2012;13:2026–45.
Birlik M, Akar S, Gurler O, et al. Prevalence of primary Sjogren’s syndrome in Turkey: a population based epidemiological study. Int J Clin Pract. 2009;63(6):954–61.
Trontzas PI, Andrianakos AA. Sjogren’s syndrome: a population based study of prevalence in Greece. The ESORDIG study. Ann Rheum Dis. 2005;64(8):1240–1.
Bartunkova J, Sediva A, Vencovsky J, Tesar V. Primary Sjogren’s syndrome (pSS) in children and adolescents: proposal for diagnostic criteria. Rheumatology. 1999;17:381–6.
Bossini N, Savoldi S, Franceschini F, et al. Clinical and morphological features of kidney involvement in primary Sjogren’s syndrome. Nephrol Dial Transplant. 2001;16:2328–36.
Bogdanovic R, Jovanovic GB, Putnik J, Stajic N, Paripovic A. Renal involvement in primary Sjogren syndrome of childhood: case report and literature review. Mod Rheumatol. 2013;23:182–9.
Bagga Arvind, Sinha Aditi. Evaluation of renal tubular acidosis. Indian J Pediatr. 2007;74(7):679–86.
Kobayashi I, Furuta H, Tame A, Kawamura N, Kojima K, Endoh M, Okano M, Sakiyama Y. Complications of childhood Sjogren syndrome. Eur J Pediatr. 1996;155:890–4.
Zeher M, Horvath IF, Szanto A, Szodoray P. Autoimmune thyroid diseases in a large group of Hungarian patients with primary Sjogren’s syndrome. Thyroid. 2009;19:39–45.
Caramaschi Paola, Biasi Domenico, Caimmi Cristian, Scambi Cinzia, Pieropan Sara, Barausse Giovanni, Adami Silvano. The co-occurrence of Hashimoto thyroiditis in primary Sjogren’s syndrome defines a subset of patients with milder clinical phenotype. Rheumatol Int. 2013;33:1271–5.
Pessler F, Emery H, Dai L, et al. The spectrum of renal tubular acidosis in pediatric Sjogren syndrome. Rheumatology. 2006;45:85–91.
Ramos-Casals M, Tzioufas AG, Stone JH, Sisó A, Bosch X. Treatment of primary Sjögren syndrome: a systematic review. JAMA. 2010;304(4):452–60.
Devauchelle-Pensec V, Mariette X, Jousse-Joulin S, Jean-Marie B, Perdriger A, Puéchal X et. al. Treatment of primary Sjo¨gren syndrome with rituximab :a randomized trial. Ann Intern Med. 2014;160:233–242.
Maripuri S, Grande JP, Osborn TG, et al. Renal involvement in primary Sjogren syndrome: a clinicopathologic study. Clin J Am Soc Nephrol. 2009;4:1423–31.
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Agarwal, A., Kumar, P. & Gupta, N. Pediatric Sjogren syndrome with distal renal tubular acidosis and autoimmune hypothyroidism: an uncommon association. CEN Case Rep 4, 200–205 (2015). https://doi.org/10.1007/s13730-015-0169-y
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DOI: https://doi.org/10.1007/s13730-015-0169-y