eMedicine Specialties > Pediatrics: General Medicine > Rheumatology

Sjogren Syndrome: Follow-up

Author: Eyal Muscal, MD, Assistant Professor, Section of Pediatric Rheumatology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital
Coauthor(s): Marietta Morales de Guzman, MD, Assistant Professor, Department of Pediatrics, Baylor College of Medicine; Consulting Staff, Section of Pediatric Rheumatology, Department of Pediatrics, Texas Children's Hospital, Ben Taub General Hospital; Lawrence K Jung, MD, Chief, Division of Pediatric Rheumatology, Children's National Medical Center; Catherine M Flaitz, DDS, MS, Professor of Oral and Maxillofacial Pathology and Pediatric Dentistry, Department of Diagnostic Sciences, University of Texas Health Sciences Center at Houston, Dental Branch
Contributor Information and Disclosures

Updated: Sep 18, 2009

Follow-up

Further Inpatient Care

  • Hospitalization, significant morbidities, and use of immunosuppressive medications are dictated by systemic extraglandular features of Sjögren syndrome.
  • Overlapping symptoms of systemic lupus erythematosus (SLE) include cerebritis, nephritis, and severe Raynaud phenomenon. Severe systemic manifestations may also develop without overt features of other autoimmune disorders.

Further Outpatient Care

  • Routine follow-up care by a rheumatologist, ophthalmologist, and dentist

Deterrence/Prevention

  • Caries prevention
    • Personal dental plaque measures include twice-daily cleaning of the teeth with a toothbrush, using a fluoride-containing dentifrice, and daily use of dental floss; increase the number of professional cleanings to 3-4 times a year if carious lesions develop.
    • Daily home use of topical fluorides, especially gel or toothpaste that contains 1.1% sodium fluoride or remineralizing gel with 0.05% sodium fluoride, sodium phosphate, and calcium carbonate, is recommended.
    • If the patient has severe xerostomia, use custom fluoride trays or carriers to apply the topical fluorides.
    • Use chlorhexidine gluconate oral rinse concurrently for 2-week periods when high numbers of Streptococcus mutans are found in the saliva (>1 X 106/mL saliva).
    • Limit the intake of sugary food and beverages between meals. Use sweetener alternatives, if tolerated, such as aspartame, saccharin, sorbitol, and xylitol.
  • Prevention of oral mucosal lesions
    • Chapped lips - Repeated use of water- or lanolin-based lip moisturizers (Avoid lip products that are medicated with menthol or phenol because they cause further drying.)
    • Traumatic erosions and ulcers - Frequent hydration and the use of artificial saliva or oral moisturizing agents, especially under removable oral prostheses (see Diet)
    • Oropharyngeal candidiasis - Good oral hygiene, frequent oral hydration and lubrication, and nightly removal and cleaning of dental prostheses (Intermittent use of topical or systemic antifungal agents may be necessary to prevent recurrent infection. If topical antifungal agents are used, consultation with a compounding pharmacist is recommended in order to formulate sucrose-free suspensions or lozenges.)

Complications

  • Adult patients have a small risk of developing lymphoma and other malignancies. These risks are unknown in children.

Prognosis

  • Patients with primary Sjögren syndrome usually have a good prognosis unless severe extraglandular manifestations appear. The prognosis of secondary Sjögren syndrome depends on the primary autoimmune disorder.

Patient Education

Miscellaneous

Medicolegal Pitfalls

  • Failure to diagnose and, thus, improve impairments of quality of life caused by sicca syndrome
  • Failure to recognize that adult patients have a small risk of developing lymphoma and other malignancies
  • Failure to review medication risks carefully with the patient and family prior to treatment with any new medication
  • Failure to diagnose systemic extraglandular manifestations of Sjögren syndrome or presence of a primary autoimmune disorder (eg, systemic lupus erythematosus [SLE]) that requires immunosuppressive agents

Special Concerns

  • Many of the symptoms associated with this disorder can seriously impair an individual's quality of life. In addition to sicca syndrome, concerns about facial appearance, depression, chronic fatigue, and joint pain must be addressed. Parotid enlargement and weight gain (if corticosteroids are used to manage the disease) may be problematic in an adolescent.
  • Close attention must be paid to emotional and cognitive functioning of the adolescent coping with a chronic disease.
 


More on Sjogren Syndrome

Overview: Sjogren Syndrome
Differential Diagnoses & Workup: Sjogren Syndrome
Treatment & Medication: Sjogren Syndrome
Follow-up: Sjogren Syndrome
Multimedia: Sjogren Syndrome
References
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References

  1. Fox RI. Sjogren's syndrome. Lancet. Jul 23-29 2005;366(9482):321-31. [Medline].

  2. Jonsson R, Haga H-J, Gordon TP. Sjogren's syndrome. In: Koopman's Textbook of Arthritis and Allied Health Conditions. 14th ed. Philadelphia, PA: Lippincott, Williams, & Wilkins; 2001:1736-59.

  3. Cassidy JT, Petty RE, Laxer RM. Overlap syndromes. In: Textbook of Pediatric Rheumatology. 5th ed. 2005:486-89.

  4. Ramos-Casals M, Font J. Primary Sjogren's syndrome: current and emergent aetiopathogenic concepts. Rheumatology (Oxford). Nov 2005;44(11):1354-67. [Medline].

  5. Kassan SS, Moutsopoulos HM. Clinical manifestations and early diagnosis of Sjögren syndrome. Arch Intern Med. Jun 28 2004;164(12):1275-84. [Medline].

  6. Civilibal M, Canpolat N, Yurt A, et al. A child with primary Sjogren syndrome and a review of the literature. Clin Pediatr (Phila). Oct 2007;46(8):738-42. [Medline].

  7. Reveille JD, Arnett FC. The immunogenetics of Sjogren's syndrome. Rheum Dis Clin North Am. Aug 1992;18(3):539-50. [Medline].

  8. Cimaz R, Casadei A, Rose C, et al. Primary Sjogren syndrome in the paediatric age: a multicentre survey. Eur J Paediatr. 2003;162 (10):661-5. [Medline].

  9. Singer NG, Tomanova-Soltys I, Lowe R. Sjogren's syndrome in childhood. Curr Rheumatol Rep. Apr 2008;10(2):147-55. [Medline].

  10. DeGuzman M, Fishman MA, Lewis RA, et al. Chronic neurologic disease with visual, gait and bladder problems in a male teenager. J Pediatr. 1998;132:742-47. [Medline].

  11. Kobayashi I, Furuta H, Tame A, et al. Complications of childhood Sjogren syndrome. Eur J Pediatr. Oct 1996;155(10):890-4. [Medline].

  12. Anaya JM, Ogawa N, Talal N. Sjogren's syndrome in childhood. J Rheumatol. Jun 1995;22(6):1152-8. [Medline].

  13. Ramos-Casals M, Solans R, Rosas J, Camps MT, Gil A, Del Pino-Montes J. Primary Sjogren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine (Baltimore). Jul 2008;87(4):210-9. [Medline].

  14. 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. Nov 2005;32(11):2225-32. [Medline].

  15. Nakamura Y, Wakamatsu E, Matsumoto I, et al. High prevalence of autoantibodies to muscarinic-3 acetylcholine receptor in patients with juvenile-onset Sjogren syndrome. Ann Rheum Dis. Jan 2008;67(1):136-7. [Medline].

  16. Arkfeld DG. The potential utility of B cell-directed biologic therapy in autoimmune diseases. Rheumatol Int. Jan 2008;28(3):205-15. [Medline].

  17. Stiller M, Golder W, Döring E, Kliem K. Diagnostic value of sialography with both the conventional and digital subtraction techniques in children with primary and secondary Sjogren's syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. Nov 1999;88(5):620-7. [Medline].

  18. Fox PC, Brennan M, Pillemer S, Radfar L, Yamano S, Baum BJ. Sjogren's syndrome: a model for dental care in the 21st century. J Am Dent Assoc. Jun 1998;129(6):719-28. [Medline].

  19. Rigante D, Inzitari R, Carone M, et al. Correspondence between clinical improvement and proteomic changes of the salivary peptide complex in a child with primary Sjogren syndrome. Rheumatol Int. Jun 2008;28(8):801-6. [Medline].

  20. Bartunkova J, Sediva A, Vencovsky J, Tesar V. Primary Sjogren's syndrome in children and adolescents: proposal for diagnostic criteria. Clin Exp Rheumatol. May-Jun 1999;17(3):381-6. [Medline].

  21. van der Reijden WA, Vissink A, Veerman EC, Amerongen AV. Treatment of oral dryness related complaints (xerostomia) in Sjogren's syndrome. Ann Rheum Dis. Aug 1999;58(8):465-74. [Medline].

  22. Athreya BH, Norman ME, Myers AR, South MA. Sjogren's syndrome in children. Pediatrics. Jun 1977;59(6):931-8. [Medline].

  23. Bell M, Askari A, Bookman A, et al. Sjogren's syndrome: a critical review of clinical management. J Rheumatol. Sep 1999;26(9):2051-61. [Medline].

  24. Chudwin DS, Daniels TE, Wara DW, et al. Spectrum of Sjogren syndrome in children. J Pediatr. Feb 1981;98(2):213-7. [Medline].

  25. Deprettere AJ, Van Acker KJ, De Clerck LS, et al. Diagnosis of Sjogren's syndrome in children. Am J Dis Child. Nov 1988;142(11):1185-7. [Medline].

  26. Fox RI, ed. Sjogren's syndrome. Vol 18. 3rd ed. 1992.

  27. Fox RI, Michelson P. Approaches to the treatment of Sjogren's syndrome. J Rheumatol Suppl. Dec 2000;61:15-21. [Medline].

  28. Garcia-Carrasco M, Ramos-Casals M, Rosas J, et al. Primary Sjogren syndrome: clinical and immunologic disease patterns in a cohort of 400 patients. Medicine (Baltimore). Jul 2002;81(4):270-80. [Medline].

  29. Leerdam CM, Martin HC, Isaacs D. Recurrent parotitis of childhood. J Paediatr Child Health. 2005;41 (12):631-4. [Medline].

  30. Manoussakis MN, Georgopoulou C, Zintzaras E, et al. Sjogren's syndrome associated with systemic lupus erythematosus: clinical and laboratory profiles and comparison with primary Sjogren's syndrome. Arthritis Rheum. Mar 2004;50(3):882-91. [Medline].

  31. McGuirt WF Jr, Whang C, Moreland W. The role of parotid biopsy in the diagnosis of pediatric Sjogren syndrome. Arch Otolaryngol Head Neck Surg. Nov 2002;128(11):1279-81. [Medline].

  32. Nikitakis NG, Rivera H, Lariccia C, et al. Primary Sjogren syndrome in childhood: report of a case and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. Jul 2003;96(1):42-7. [Medline].

  33. Pessler F, Emery H, Dai L, et al. The spectrum of renal tubular acidosis in paediatric Sjogren syndrome. Rheumatology (Oxford). Jan 2006;45(1):85-91. [Medline].

  34. Pessler F, Monash B, Rettig P, Forbes B, Kreiger PA, Cron RQ. Sjogren syndrome in a child: favorable response of the arthritis to TNFalpha blockade. Clin Rheumatol. Sep 2006;25(5):746-8. [Medline].

  35. Ramos-Casals M, Cervera R, Font J, et al. Young onset of primary Sjogren's syndrome: clinical and immunological characteristics. Lupus. 1998;7(3):202-6. [Medline].

  36. Ramos-Casals M, Tzioufas AG, Font J. Primary Sjogren's syndrome: new clinical and therapeutic concepts. Ann Rheum Dis. Mar 2005;64(3):347-54. [Medline].

  37. Rhodus NL. Sjogren's syndrome. Quintessence Int. Oct 1999;30(10):689-99. [Medline].

  38. Siamopoulou-Mavridou A, Drosos AA, Andonopoulos AP. Sjogren syndrome in childhood: report of two cases. Eur J Pediatr. Apr 1989;148(6):523-4. [Medline].

  39. Stiller M, Golder W, Doring E, Biedermann T. Primary and secondary Sjogren's syndrome in children--a comparative study. Clin Oral Investig. Sep 2000;4(3):176-82. [Medline].

  40. Suzuki K, Matsumoto M, Nakashima M, et al. Effect of cevimeline on salivary components in patients with Sjogren syndrome. Pharmacology. May 2005;74(2):100-5. [Medline].

  41. Tzioufas AG, Moutsopoulos HM. Sjogren syndrome. In: Klippel J, Dieppe P, eds. Rheumatology. 2nd ed. St. Louis, MO: Mosby; 1998:7.32.1-7.32.12.

  42. Williams FM, Cohen PR, Jumshyd J, Reveille JD. Prevalence of the diffuse infiltrative lymphocytosis syndrome among human immunodeficiency virus type 1-positive outpatients. Arthritis Rheum. May 1998;41(5):863-8. [Medline].

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Further Reading

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Keywords

Sjogren syndrome, Sjögren's syndrome, Sjögren syndrome, Sicca syndrome, keratoconjunctivitis, xerostomia, polyarthritis, parotitis, salivary gland enlargement, recurrent parotitis, autoimmune exocrinopathy, systemic lupus erythematosus, SLE, rheumatoid arthritis, scleroderma, biliary cirrhosis, lymphoproliferative disease, non-Hodgkin lymphoma, Waldenström macroglobulinemia, B-cell lymphoma, keratoconjunctivitis, Raynaud phenomenon, Epstein-Barr virus, EBV, HIV, cytomegalovirus, treatment, diagnosis

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Contributor Information and Disclosures

Author

Eyal Muscal, MD, Assistant Professor, Section of Pediatric Rheumatology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital
Eyal Muscal, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Rheumatology, and Clinical Immunology Society
Disclosure: Nothing to disclose.

Coauthor(s)

Marietta Morales de Guzman, MD, Assistant Professor, Department of Pediatrics, Baylor College of Medicine; Consulting Staff, Section of Pediatric Rheumatology, Department of Pediatrics, Texas Children's Hospital, Ben Taub General Hospital
Marietta Morales de Guzman, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Rheumatology, and Texas Pediatric Society
Disclosure: Nothing to disclose.

Lawrence K Jung, MD, Chief, Division of Pediatric Rheumatology, Children's National Medical Center
Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Catherine M Flaitz, DDS, MS, Professor of Oral and Maxillofacial Pathology and Pediatric Dentistry, Department of Diagnostic Sciences, University of Texas Health Sciences Center at Houston, Dental Branch
Catherine M Flaitz, DDS, MS is a member of the following medical societies: American Academy of Oral and Maxillofacial Pathology, American Academy of Oral Medicine, American Academy of Pediatric Dentistry, American Dental Association, International Association for Dental Research, and International Association of Oral Pathologists
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

David D Sherry, MD, Director, Clinical Rheumatology, Attending Physician, Pain Management, The Children's Hospital of Philadelphia; Professor of Pediatrics, University of Pennsylvania
David D Sherry, MD is a member of the following medical societies: American College of Rheumatology and American Pain Society
Disclosure: Nothing to disclose.

CME Editor

Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine
Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine
Disclosure: Baxter Honoraria Consulting

 
 
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