A 16-year old girl presented with rapid onset of muscular weakness and a history of severe dysphagia, dysphonia and significant wasting. On examination, she was dystrophic (BMI 15.7) and had signs of myopathy. Laboratory findings confirmed myopathy (CPK 106.4 microkat/L (6384 IU/L), AST 2.86 microkat/L (171.6 IU/L), myoglobin 1582 microg/L). There was profound hypokalaemia (S-K 1.8 mmol/L) suggesting hypokalaemic paralysis. Diagnosis of distal renal tubular acidosis (dRTA) was based on combination of hyperchloremic metabolic acidosis, severe hypokalaemia, high urinary pH and positive value of urinary anion gap. There was evidence of other signs of renal tubular impairment (urinary beta-2-microglobulin 213 mg/L, glomerulotubular proteinuria 1.01g/24h). Autoimmune tests (rheumatoid factor, antinuclear antibodies, autoantibodies to Ro/SSA and La/SSB) together with symptoms of xerostomia with swallowing difficulties and atrophic glossitis suggested primary Sjogren's syndrome (SS) as the underlying cause of dRTA. The renal biopsy confirmed chronic tubulo-interstitial nephritis compatible with this diagnosis. Full recovery of muscle weakness and hypokalaemia and acidosis followed after potassium and alkali replacement therapy. Corticosteroids were administered with subsequent addition of cyclosporine A because of disease activity. In conclusion, primary SS is a rare diagnosis in childhood and adolescence and should be considered in patients presenting with hypokalaemic paralysis, as this might be due to dRTA, even in the absence of apparent sicca syndrome.

Department of Paediatrics and Faculty of Medicine and Teaching Hospital Hradec Kralove Charles University Prague Hradec Kralove Czech Republic

Zobrazit více v PubMed

Ahlawat Sushil K, Sachdev A. Classic diseases revisited: Hypokalemic paralysis. Postgrad Med J. 1999;75:193–197. PubMed PMC

Lin SH, Lin YF, Halperin ML. Hypokalaemia nad paralysis. Q J Med. 2001;94:133–139. PubMed

Bagga A, Bajpai A, Menon S. Approach to renal tubular disorders. Indian J Pediatr. 2005;72:771–776. PubMed

Ramos-Casals M, Font J. Primary Sjogren's syndrome: current and emergent aethiopathogenic concepts. Rheumatology. 2005;44:1354–1367. PubMed

Houghton K, Malleson P, Cabral D, Petty R, Tucker L. Primary Sjogren's syndrome in children and adolescents: Are proposed diagnostic criteria applicable? J Rheumatol. 2005;32:2225–2232. PubMed

Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, Daniels TE, Fox PC, Fox RI, Kassan SS, Pillemer SR, Talal N, Weisman MH. European Study Group on Classification Criteria for Sjögren's Syndrome. Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002;61:554–558. PubMed PMC

Bartunkova J, Sedivá A, Vencovský J, Tesar V. Primary Sjögren's syndrome in children and adolescents: proposal for diagnostic criteria. Clin Exp Rheumatol. 1999;17:381–386. PubMed

Cimaz R, Casadei A, Rose C, Bartunkova J, Sediva A, Falcini F, Picco P, Taglietti M, Zulian F, Ten Cate R, Sztajnbok FR, Voulgari PV, Drosos AA. Primary Sjögren syndrome in the paediatric age: multicenter survey. Eur J Ped. 2003;162:661–665. PubMed

Pessler F, Emery H, Dai L, Wu Y-M, Monash B, Cron RQ, Pradhan M. The spectrum of renal tubular acidosis in paediatric Sjögren syndrome. Rheumatology. 2006;45:85. PubMed

Bossini N, Savoldi S, Franceschini F, Mombelloni S, Baronio M, et al. Clinical and morphological features of kidney involvement in primary Sjögren's syndrome. Nephrol Dial Transplant. 2001;16:2328–2336. PubMed

Pun KK, Wong CK, Tsui EY, Tam SC, Kung AW, Wang CC. Hypokalemic periodic paralysis due to the Sjögren syndrome in Chinese patients. Ann Intern Med. 1989;110:405–406. PubMed

Hattori N, Hino M, Ishihara T, Moridera K, Ikekubo K, Kurahachi H. Hypokalemic paralysis associated with distal renal tubular acidosis. Intern Med. 1992;31:662–665. PubMed

Chang YC, Huang CC, Chiou YY, Yu CY. Renal tubular acidosis complicated with hypokalemic periodic paralysis. Pediatr Neurol. 1995;13:52–54. PubMed

al Jubouri MA, Jones S, Macmillan R, Harris C, Griffiths RD. Hypokalemic paralysis revealing Sjogren syndrome in an elderly man. J Clin Pathol. 1999;52:157–158. PubMed PMC

Soy M, Pamuk ON, Gerenli M, Çelik Y. A primary Sjögren's syndrome patient with distal renal tubular acidosis, who presented with symptomes of hypokalemic periodic paralysis. Rheumatol Int. 2005;26:86–89. PubMed

Cheng CJ, Chiu JS, Chen CC, Lin SH. Unusual cause of hypokalemic paralysis in aged men: Sjögren syndrome. South Med J. 2005;98:1212–1215. PubMed

Kawashima M, Amano T, Morita Y, Yamamura M, Makino H. Hypokalemic paralysis and osteomalacia secondary to renal tubular acidosis in a case with primary Sjogren's syndrome. Mod Rheumatol. 2006;16:48–51. PubMed

Ohlsson V, Strike H, James-Ellison M, Tizard EJ, Ramanam AV. Renal tubular acidosis, arthritis and autoantibodies: primary Sjogren syndrome in childhood. Rheumatology. 2006;45:238–240. PubMed

Morovic-Vergles J, Galesic K, Vergles D. Primary Sjogren's syndrome presenting as hypokalemic paralysis. Ann Saudi Med. 2007;27:125–127. PubMed PMC

Taylor I, Parsons M. Hypokalemic paralysis revealing Sjögren's syndrome. J Clin Neurosci. 2004;11:319–321. PubMed

Saeki Y, Ohshima S, Ishida T, Umeshita-Sasai M, Nishioka K, Yamaguchi N, M Suemura M. Remission of the renal involvement in a patient with primary Sjögren's syndrome (SS) after pulse high-dose corticosteroid infusion therapy. Clin Rheumatol. 2001;20:225–228. PubMed

Juvenile Primary Sjögren Syndrome in a 15-Year-Old Boy with Renal Involvement: A Case Report and Review of the Literature