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A Pediatric Case of Primary Sj$\ddot{o}$gren's Syndrome Associated with Nephrogenic Diabetes Insipidus and Renal Tubular Acidosis

신성요붕증과 신세뇨관산증을 동반한 일차성 쇠그렌 증후군의 1례

  • Choi, Jong Won (Department of Pediatrics, The Catholic University of Korea, College of Medicine) ;
  • Jung, You Jin (Department of Pediatrics, The Catholic University of Korea, College of Medicine) ;
  • Suh, Jin Soon (Department of Pediatrics, The Catholic University of Korea, College of Medicine) ;
  • Park, So Hyun (Department of Pediatrics, The Catholic University of Korea, College of Medicine) ;
  • Koh, Dae Kyun (Department of Pediatrics, The Catholic University of Korea, College of Medicine)
  • 최종원 (가톨릭대학교 의과대학 소아과학교실) ;
  • 정유진 (가톨릭대학교 의과대학 소아과학교실) ;
  • 서진순 (가톨릭대학교 의과대학 소아과학교실) ;
  • 박소현 (가톨릭대학교 의과대학 소아과학교실) ;
  • 고대균 (가톨릭대학교 의과대학 소아과학교실)
  • Received : 2012.03.18
  • Accepted : 2012.06.20
  • Published : 2012.10.31

Abstract

Sj$\ddot{o}$gren's syndrome (SS) is an autoimmune disorder primarily affecting the salivary and lacrimal glands. In addition, extra-glandular manifestations involving the lungs, liver, kidneys, pancreas, skin and central nervous system were reported in patients with SS. These extra-glandular manifestations are not rare in adult patient, but are very rare in pediatric SS. Renal manifestations are relatively common in adult SS, but are rarely reported in childhood SS. We experienced a girl with primary SS manifested with nephrogenic diabetes insipidus and renal tubular acidosis.

쇠그렌 증후군은 주로 침샘과 눈물샘을 침범하는 자가 면역 질환으로, 폐나 간, 콩팥, 췌장, 피부, 신경계 등의 다른 장기 역시 침범하여 임상 증상을 나타내기도 한다. 성인의 경우 이런 다른 장기와 관련된 증상은 드물지 않으나 소아에서는 매우 드물다고 알려져 있다. 특히 콩팥을 침범한 쇠그렌 증후군은 성인의 경우 비교적 흔하지만 소아는 매우 드문데, 이에 본 저자들은 신성 요붕증과 신세뇨관 산증을 주 증상으로 진단한 소아의 일차성 쇠그렌 증후군 1례를 경험하였기에 보고한다.

Keywords

References

  1. Carsons S. Sjogren's syndrome. In: Firestein GS, Budd RC, Harris ED Jr., Mclnnes IB, Ruddy S, Sergent JS, editors. Textbook of Rheumatology. 8 th ed. Philadelphia:Elsevier Saunders, 2008:1149-68.
  2. Garcia-Carrasco M, Ramos-Casals M, Rosas J, Pallares L, Calvo-Alen J, Cervera R, et al. Primary Sjogren's syndrome: clinical and immunologic disease patterns in a cohort of 400 patients. Medicine 2002;81:270-80. https://doi.org/10.1097/00005792-200207000-00003
  3. Kobayashi I, Furuta H, Tame A, Kawamura N, Kojima K, Endoh M, et al. Complications of childhood Sjögren's syndrome. Eur J Pediatr 1996;155:890-4. https://doi.org/10.1007/BF02282840
  4. Goules A, Masouridi S, Tzioufas AG, Ioannidis JP, Skopouli FN, Moutsopoulos HM. Clinically significant and biopsy-documented renal involvement in primary Sjögren's syndrome. Medicine 2000;79:241-9. https://doi.org/10.1097/00005792-200007000-00005
  5. Ohtani H, Imai H, Kodama T, Hamai K, Komatsuda A, Wakui H, et al. Severe hypokalaemia and respiratory arrest due to renal tubular acidosis in a patient with Sjögren's syndrome. Nephrol Dial Transplant 1999;14:2201-3. https://doi.org/10.1093/ndt/14.9.2201
  6. Cimaz R, Casadei A, Rose C, Bartunkova J, Sediva A, Falcini F, et al. Primary Sjögren's syndrome in the paediatric age: a multicentre survey. Eur J Pediatr 2003;162:661-5. https://doi.org/10.1007/s00431-003-1277-9
  7. Jung SK, Park KH, Yim HE, Yoo KH , Hong YS, Lee JW, et al. Primary Sjögren's syndrome with mesangial proliferative glomerulonephritis and IgA deposits in a child. Pediatr Nephrol 2010;25:567-8. https://doi.org/10.1007/s00467-009-1358-8
  8. Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, 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;61:554-8. https://doi.org/10.1136/ard.61.6.554
  9. Tsokos M, Lazarou SA, Moutsopoulos HM. Vasculitis in primary Sjogren's syndrome. Histologic classification and clinical presentation. Am J Clin Pathol 1987;88:26-31. https://doi.org/10.1093/ajcp/88.1.26
  10. Shiozawa S, Shiozawa K, Shimizu S, Nakada M, Isobe T, Fujita T. Clinical studies of renal disease in Sjögren's syndrome. Ann Rheum Dis 1987;46:768-72. https://doi.org/10.1136/ard.46.10.768
  11. Pessler F, Emery H, Dai L, Wu YM, Monash B, Cron RQ, et al. The spectrum of renal tubular acidosis in paediatric Sjögren's syndrome. Rheumatology 2006;45:85-91. https://doi.org/10.1093/rheumatology/kei110
  12. Shearn MA, Tu WH. Nephrogenic diabetes insipidus and other defects of renal tubular function in Sjögren's syndrome. Am J Med 1965;39:312-8. https://doi.org/10.1016/0002-9343(65)90057-4
  13. Bogdanovic R, Basta-Jovanovic G, Putnik J, Stajic N, Paripovic A. Renal involvement in primary Sjogren syndrome of childhood: case report and literature review. Mod Rheumatol. 2012:Apr 7. [Epub ahead of print].
  14. Cohen EP, Bastani B, Cohen MR, Kolner S, Hemken Ph, Gluck SL. Absence of H+-ATPase in cortical collecting tubules of a patient with Sjögren's syndrome and distal renal tubular acidosis. J Am Soc Nephrol 1992;3:264-70.
  15. Janson RW, Arend PW. Renal disorders associated with systemic sclerosis, rheumatoid arthritis, Sjogren's syndrome, and polymyositis-dermatomyositis. In: Schrier RW, editor. Diseases of the kidney and urinary tract 8th ed. Philadelphia: Lipincott williams & wilkins, 2007;1712-13.
  16. Ito S, Kamei K, Ikoma M. Primary Sjögren syndrome that developed after IgA nephropathy. Pediatr Nephrol 2010;25: 1579-80. https://doi.org/10.1007/s00467-010-1489-y
  17. Talal N, Zisman E, Schur PH. Renal tubular acidosis, glomerulonephritis and immunologic factors in Sjögren's syndrome. Arthritis Rheum 1968;11:774-86. https://doi.org/10.1002/art.1780110607
  18. Ohlsson V, Strike H, James-Ellison M, Tizard EJ, Ramanan AV. Renal tubular acidosis, arthritis and autoantibodies: primary Sjögren's syndrome in childhood. Rheumatology 2006;45: 238-40. https://doi.org/10.1093/rheumatology/kei175
  19. Khanna A. Acquired nephrogenic diabetes insipidus. Semin Nephrol 2006;26:244-8. https://doi.org/10.1016/j.semnephrol.2006.03.004
  20. Garofeanu CG, Weir M, Rosas-Arellano MP, Henson G, Garg AX, Clark WF. Causes of reversible nephrogenic diabetes insipidus: a systematic review. Am J Kidney Dis 2005;45:626-37. https://doi.org/10.1053/j.ajkd.2005.01.008