Journal of Oral Medicine and Pain

Korean Academy of Orofacial Pain and Oral Medicine (대한안면통증구강내과학회)
권호 표지

Rational Diagnosis and Management of Sjogren's Syndrome

쉐그렌 증후군의 합리적 진단 및 관리

  • Tae, Il-Ho (Department of Oral Diagnosis & Oral Medicine, Dental Hospital, Yonsei University, College of Dentistry) ;
  • Kwon, Jeong-Seung (Department of Oral Diagnosis & Oral Medicine, Dental Hospital, Yonsei University, College of Dentistry) ;
  • Jeon, Young-Mi (Department of Oral Diagnosis & Oral Medicine, Dental Hospital, Yonsei University, College of Dentistry) ;
  • Choi, Jong-Hoon (Department of Oral Diagnosis & Oral Medicine, Dental Hospital, Yonsei University, College of Dentistry) ;
  • Shim, Woo-Hyun (Department of Oral Diagnosis & Oral Medicine, Dental Hospital, Yonsei University, College of Dentistry) ;
  • Ahn, Hyung-Joon (Department of Oral Diagnosis & Oral Medicine, Dental Hospital, Yonsei University, College of Dentistry)
  • 태일호 (연세대학교 치과대학 구강내과학교실) ;
  • 권정승 (연세대학교 치과대학 구강내과학교실) ;
  • 전영미 (연세대학교 치과대학 구강내과학교실) ;
  • 최종훈 (연세대학교 치과대학 구강내과학교실) ;
  • 심우현 (연세대학교 치과대학 구강내과학교실) ;
  • 안형준 (연세대학교 치과대학 구강내과학교실)
  • Published : 2007.12.30
Download PDF
⟨ Previous Next ⟩

Abstract

Sjogren's syndrome is a chronic autoimmune disorder of the exocrine glands with associated lymphocytic infiltrates of the affected glands. Primary SS presents alone as xerostomia, keratoconjunctivitis sicca and patotid gland enlargement, secondary SS occurs in connection with other autoimmune disorder such as rheumatitoid arthritis, systemic lupus erythematosus, or progressive systemic sclerosis. Among many other systemic complication, lymphoma is considered seriously. Patient with SS had a 44 times higher relative risk of lymphoma, and clinically identifiable lymphoma occurs in approximately 5% of patients with SS. So, patients with SS should be closely monitored. In dental office, diagnosis of SS is important in view of high risk of lymphoma. When a dentist diagnose and manage dry mouth, he or she should consider possibility of SS all the times and have knowledge of diagnostic criteria of SS.

쉐그렌 증후군은 만성 자가 면역 질환으로 특히 눈물샘과 침샘에 영향을 주어 구강건조증, 건성각결막염, 이하선 확대 등을 나타낸다. 일반적으로, 특발성으로 발생하여 쉐그렌 증후군이 단독으로 나타나는 원발성 쉐그렌 증후군과 류마티스 관절염, 전신성 홍반성 낭창 등과 같은 다른 자가 면역 질환과 함께 나타나는 이차성 쉐그렌 증후군으로 분류할 수 있다. 쉐그렌 증후군의 경우, 구강 건조감으로 인한 불편감을 호소하는 대신, 불쾌한 맛, 이하선 부위의 붓는 느낌 등 구강건조증 이외의 증상을 호소하기도 한다. 따라서 구강건조증을 호소하는 환자에 있어서만 쉐그렌 증후군을 감별진단에서 고려할 것이 아니라, 쉐그렌 증후군의 관련 소견 중 구강건조증 이외의 증상이나 징후에 대해서도 충분히 주목하여, 관련 증상이나 징후를 보이는 경우 쉐그렌 증후군 관련 검사를 고려하고 시행하는 것이 필요하며, 2002년 수정된 쉐그렌 증후군 진단 기준에 부합되는 검사들을 통해 조기에 진단을 하는 것이 필수적일 것으로 사료된다. Manthorpe등은 처음 쉐그렌 증상이 발생한 때로부터 진단이 될 때까지 평균 10년이라는 긴 시간이 소요된다고 보고였는데, 이는 쉐그렌 증후군 진단이 조기에 잘 이루어지지 않는다는 것을 반영하는 것임을 알 수 있다. Kassan등에 의하면, 쉐그렌 증후군의 여러 전신적 증상 중 림프종의 발생 정도는 건강한 사람들에 비해 쉐그렌 증후군을 갖고 있는 환자의 경우 44배정도 높은 것으로 보고되고 있다. 따라서 쉐그렌 증후군 환자의 정확하고 신속한 진단은 쉐그렌 증후군과 관련하여 발생될 수 있는 합병증등을 예방, 관리하는데 있어서 중요한 의미를 지닌다고 할 수 있다.

Keywords

References

  1. Kassan SS, Moutsopoulos HM. Clinical manifestations and early diagnosis of Sjögren syndrome. Arch Intern Med 2004;164(12):1275-1284 https://doi.org/10.1001/archinte.164.12.1275
  2. Vitali C, Bombardieri S, Jonsson R et al. 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(6):554-558 https://doi.org/10.1136/ard.61.6.554
  3. Dafni UG, Tzioufas AG, Staikos P et al. Prevalence of Sjögren's syndrome in a closed rural community. Ann Rheum Dis 1997;56(9):521-525 https://doi.org/10.1136/ard.56.9.521
  4. Daniels TE. Labial salivary gland biopsy in Sjögren's syndrome. Assessment as a diagnostic criterion in 362 suspected cases. Arthritis Rheum 1984;27(2):147-156 https://doi.org/10.1002/art.1780270205
  5. Daniels TE. Salivary histopathology in diagnosis of Sjogren's syndrome. Scand J Rheumatol Suppl 1986;61:36-43
  6. Manthorpe R, Asmussen K, Oxholm P. Primary Sjögren's syndrome: diagnostic criteria, clinical features, and disease activity. J Rheumatol 1997;24(suppl):8-11
  7. Kassan SS. Managing dry eyes and dry mouth in Sjogren's syndrome. Am J Manag Care 2001;7 (Suppl):S444-450
  8. Koopman WJ, Moreland LW. Arthritis and Allied Conditions. 13th ed., Philadelphia, 1997, Williams & Wilkins, pp.561-1580
  9. Al-Hashimi I, Khuder S, Haghighat N et al. Frequency and predictive value of the clinical manifestations in Sjogren's syndrome. J Oral Pathol Med 2001;30:1-6 https://doi.org/10.1034/j.1600-0714.2001.300101.x
  10. Franquet T, Diaz C, Domingo P et al. Air trapping in primary Sjögren's syndrome: correlation of expiratory CT with pulmonary function tests. J Comput Assist Tomogr 1999;23:169-173 https://doi.org/10.1097/00004728-199903000-00002
  11. Moutsopoulos HM. Sjögren's syndrome: autoimmune epithelitis. Clin Immunol Immunopathol 1994;72:162-165 https://doi.org/10.1006/clin.1994.1123
  12. Tu WH, Shearn MA, Lee JC et al. Interstitial nephritis in Sjogren's syndrome. Ann Intern Med 1968;69: 1163-1170 https://doi.org/10.7326/0003-4819-69-6-1163
  13. Cohen EP, Bastani B, Cohen MR et al. 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;3:264-271
  14. Shearn MA, Tu W. Nephrogenic diabetes insipidus and other defects of tubular function in Sjogren's syndrome. Am J Med 1965;39:312 https://doi.org/10.1016/0002-9343(65)90057-4
  15. Wrong OM, Feest TG, Maclver AG. Immunerelated potassium-losing interstitial nephritis: a comparison with distal renal tubular acidosis. Q J Med 1993;86:513-534 https://doi.org/10.1093/qjmed/86.8.513
  16. Bloch KJ, Buchanan WW, Wohl MJ et al. Sjogren's syndrome: a clinical, pathological, and serological study in 62 cases. Medicine(Baltimore) 1965;44:187-231
  17. Bailey RR, Swainson CP. Renal involvement in Sjogren's. N Z Med J 1986;99:579-580
  18. Talal N, Zisman E, Schur PH. Renal tubular acidosis, glomerulonephritis and immunologic factors in Sjogren's syndrome. Arthritis Rheum 1968;11:774-786 https://doi.org/10.1002/art.1780110607
  19. Gemignani F, Marbini A, Pavesi G et al. Peripheral neuropathy associated with primary Sjögren's syndrome. J Neurol Neurosurg Psychiatry 1994;57: 983-986 https://doi.org/10.1136/jnnp.57.8.983
  20. Kassan SS, Thomas TL, Moutsopoulos HM et al. Increased risk of lymphoma in sicca syndrome. Ann Intern Med 1978;89:888-892 https://doi.org/10.7326/0003-4819-89-6-888
  21. Freimark B, Fantozzi R, Bone R et al. Detection of clonally expanded salivary gland lymphocytes in Sjogren's syndrome. Arthritis Rheum 1989;32:859-869
  22. Kassirer JP, Greene HL. Current Therapy in Adult Medicine. 4th ed., Baltimore, 1997, Mosby, pp.1291-1298
  23. Skopouli FN, Dafni U, Ioannidis JP et al. Clinical evolution, and morbidity and mortality of primary Sjogren's syndrome. Semin Arthritis Rheum 2000;29:296-304 https://doi.org/10.1016/S0049-0172(00)80016-5
  24. Tzioufas AG, Boumba DS, Skopouli FN et al. Mixed monoclonal cryoglobulinemia and monoclonal rheumatoid factor crossreactive idiotypes as predictive factors for the development of lymphoma in primary Sjogren's syndrome. Arthritis Rheum 1996;39:767-772 https://doi.org/10.1002/art.1780390508