eMedicine Specialties > Ophthalmology > Connective Tissue Disorders

Sjogren Syndrome: Follow-up

Author: James V Aquavella, MD, Professor of Ophthalmology, Department of Ophthalmology, University of Rochester School of Medicine, University of Rochester Eye Institute
Coauthor(s): Zoe R Williams, MD, Staff Physician, Department of Ophthalmology, University of Rochester School of Medicine, Strong Memorial Hospital; Shobha Boghani, MD, Assistant Professor, Department of Ophthalmology, Strong Memorial Hospital, University of Rochester
Contributor Information and Disclosures

Updated: Feb 19, 2008

Follow-up

Further Outpatient Care

  • Patients should receive follow-up care as needed.

Deterrence/Prevention

  • Wearing spectacles or goggles is beneficial in preventing ocular surface drying secondary to exposure.
  • The placement of air conditioning or heating ducts at home and at the workplace should be considered.
  • Avoidance of desiccating environments, such as proximity of open fireplaces or smoke-filled rooms, should be considered.
  • Systemic medications also can adversely affect the ocular surface. Reduction or elimination of such medications could decrease the drying of the ocular surface.

Complications

  • Severe dry eyes can cause corneal scarring, ulceration, infection, and even perforation.

Prognosis

  • While the prognosis is good for most patients, eyes with corneal infection, ulceration, or perforation have a much more guarded prognosis.

Patient Education

  • Patients should know to seek ophthalmic care if their eyes become red or painful, or if they develop a mucopurulent discharge.
  • For excellent patient education resources, visit eMedicine's Arthritis Center. Also, see eMedicine's patient education article Sjögren Syndrome.

Miscellaneous

Medicolegal Pitfalls

  • In any of these individuals who are treated with immunosuppression, it is extremely important that the ophthalmologist be aware of the patient's status regarding follow-up laboratory studies. It is also important for these patients to have contact with their internist and/or immunologist. This should be noted clearly on the medical record at each patient visit.
  • If nonpreserved medications and/or hydrophilic bandage lenses are used, one must always be aware of the significant potential for secondary infection. These risks should be explained to the patient prior to initiating these forms of therapy. The use of a prophylactic antibiotic is the standard of care.
  • In the presence of a significantly compromised ocular surface, there is some reluctance to use topical anesthetic and applanation tonometry. While the intraocular pressure recording may not be necessary at each particular visit, it should clearly be performed and its results indicated on the chart at periodic intervals. Additionally with the anterior segment ocular surface disorder, routine follow-up care of the posterior segment also may be compromised. Care should be taken to perform either dilated fundus examinations or, at the very least, fundus examinations without dilatation at intervals that are appropriate for the patient's age and general status.
 


More on Sjogren Syndrome

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

References

  1. Yoon KC, Jeong IY, Park YG, Yang SY. Interleukin-6 and tumor necrosis factor-alpha levels in tears of patients with dry eye syndrome. Cornea. May 2007;26(4):431-7. [Medline].

  2. Koufakis DI, Karabatsas CH, Sakkas LI, Alvanou A, Manthos AK, Chatzoulis DZ. Conjunctival surface changes in patients with Sjogren's syndrome: a transmission electron microscopy study. Invest Ophthalmol Vis Sci. Feb 2006;47(2):541-4. [Medline].

  3. Villani E, Galimberti D, Viola F, Mapelli C, Ratiglia R. The cornea in Sjogren's syndrome: an in vivo confocal study. Invest Ophthalmol Vis Sci. May 2007;48(5):2017-22. [Medline].

  4. Spiteri A, Mitra M, Menon G, Casini A, Adams D, Ricketts C, et al. Tear lipid layer thickness and ocular comfort with a novel device in dry eye patients with and without Sjögren's syndrome. J Fr Ophtalmol. Apr 2007;30(4):357-64. [Medline].

  5. Aguilar AJ, Fonseca L, Croxatto JO. Sjogren's syndrome: a comparative study of impression cytology of the conjunctiva and buccal mucosa, and salivary gland biopsy. Cornea. May 1991;10(3):203-6. [Medline].

  6. Aragona P, Bucolo C, Spinella R, Giuffrida S, Ferreri G. Systemic omega-6 essential fatty acid treatment and pge1 tear content in Sjögren's syndrome patients. Invest Ophthalmol Vis Sci. Dec 2005;46(12):4474-9. [Medline].

  7. Aragona P, Di Pietro R. Is it safe to use topical NSAIDs for corneal sensitivity in Sjögren's syndrome patients?. Expert Opin Drug Saf. Jan 2007;6(1):33-43. [Medline].

  8. Aragona P, Di Pietro R, Spinella R, Mobrici M. Conjunctival epithelium improvement after systemic pilocarpine in patients with Sjogren's syndrome. Br J Ophthalmol. Feb 2006;90(2):166-70. [Medline].

  9. Bloch KJ, Buchanan WW, Wohl MJ, Bunim JJ. Sjögren's syndrome. A clinical, pathological, and serological study of sixty-two cases. 1965. Medicine (Baltimore). Nov 1992;71(6):386-401; discussion 401-3. [Medline].

  10. Chused TM, Kassan SS, Opelz G, Moutsopoulos HM, Terasaki PI. Sjögren's syndrome association with HLA-Dw3. N Engl J Med. Apr 21 1977;296(16):895-7. [Medline].

  11. Cordero-Coma M, Anzaar F, Sobrin L, Foster CS. Systemic immunomodulatory therapy in severe dry eye secondary to inflammation. Ocul Immunol Inflamm. Mar-Apr 2007;15(2):99-104. [Medline].

  12. Daniels TE. Benign lymphoepithelial lesion and Sjogren's syndrome. In: Ellis GL, AuClair PL, eds. Surgical Pathology of the Salivary Glands. WB Saunders; 1991.

  13. Drosos AA, Skopouli FN, Costopoulos JS, Papadimitriou CS, Moutsopoulos HM. Cyclosporin A (CyA) in primary Sjögren's syndrome: a double blind study. Ann Rheum Dis. Sep 1986;45(9):732-5. [Medline].

  14. Fox RI. Sjögren's syndrome: current therapies remain inadequate for a common disease. Expert Opin Investig Drugs. Sep 2000;9(9):2007-16. [Medline].

  15. Fox RI, Konttinen Y, Fisher A. Use of muscarinic agonists in the treatment of Sjögren's syndrome. Clin Immunol. Dec 2001;101(3):249-63. [Medline].

  16. Fox RI, Pearson G, Vaughan JH. Detection of Epstein-Barr virus-associated antigens and DNA in salivary gland biopsies from patients with Sjogren's syndrome. J Immunol. Nov 15 1986;137(10):3162-8. [Medline].

  17. Fox RI, Robinson CA, Curd JG, Kozin F, Howell FV. Sjögren's syndrome. Proposed criteria for classification. Arthritis Rheum. May 1986;29(5):577-85. [Medline].

  18. Gottenberg JE, Cagnard N, Lucchesi C, Letourneur F, Mistou S, Lazure T, et al. Activation of IFN pathways and plasmacytoid dendritic cell recruitment in target organs of primary Sjögren's syndrome. Proc Natl Acad Sci U S A. Feb 21 2006;103(8):2770-5. [Medline].

  19. Jonsson R, Moen K, Vestrheim D, Szodoray P. Current issues in Sjögren's syndrome. Oral Dis. May 2002;8(3):130-40. [Medline].

  20. Lindahl G, Hedfors E, Klareskog L, Forsum U. Epithelial HLA-DR expression and T lymphocyte subsets in salivary glands in Sjögren's syndrome. Clin Exp Immunol. Sep 1985;61(3):475-82. [Medline].

  21. Lodde BM, Baum BJ, Tak PP, Illei G. Experience with experimental biological treatment and local gene therapy in Sjogren's syndrome: implications for exocrine pathogenesis and treatment. Ann Rheum Dis. Nov 2006;65(11):1406-13. [Medline].

  22. Manthrope R, Frost-Larsen K. Sjogren's syndrome: a review with emphasis on immunological features. Allergy. 1981;36:139-153.

  23. Mavragani CP, Moutsopoulos NM, Moutsopoulos HM. The management of Sjögren's syndrome. Nat Clin Pract Rheumatol. May 2006;2(5):252-61. [Medline].

  24. Miyasaka N, Seaman W, Bakshi A, Sauvezie B, Strand V, Pope R, et al. Natural killing activity in Sjögren's syndrome. An analysis of defective mechanisms. Arthritis Rheum. Aug 1983;26(8):954-60. [Medline].

  25. Mizuno Y, Hara T, Hatae K, Hirano T, Ueda K, Nakamura N, et al. Recurrent parotid gland enlargement as an initial manifestation of Sjögren syndrome in children. Eur J Pediatr. Feb 1989;148(5):414-6. [Medline].

  26. Moutsopoulos HM, Chused TM, Mann DL, Klippel JH, Fauci AS, Frank MM, et al. Sjögren's syndrome (Sicca syndrome): current issues. Ann Intern Med. Feb 1980;92(2 Pt 1):212-26. [Medline].

  27. Murillo-Lopez F, Pflugfelder SC. Disorders of tear production and the lacrimal system. In: Krachmer JH, ed. Cornea. Mosby Year Book; 1997:663-686.

  28. Pepose JS, Akata RF, Pflugfelder SC, Voigt W. Mononuclear cell phenotypes and immunoglobulin gene rearrangements in lacrimal gland biopsies from patients with Sjögren's syndrome. Ophthalmology. Dec 1990;97(12):1599-605. [Medline].

  29. Pflugfelder SC. Lacrimal gland epithelial and immunopathology of Sjogren's syndrome. In: Homma M, ed. Proceedings of the IV International Sjogren's Syndrome Symposium, Kugler. Am Steleveen; 1994.

  30. Pflugfelder SC, Crouse C, Pereira I, Atherton S. Amplification of Epstein-Barr virus genomic sequences in blood cells, lacrimal glands, and tears from primary Sjögren's syndrome patients. Ophthalmology. Aug 1990;97(8):976-84. [Medline].

  31. Pflugfelder SC, Crouse CA, Monroy D, Yen M, Rowe M, Atherton SS. Epstein-Barr virus and the lacrimal gland pathology of Sjögren's syndrome. Am J Pathol. Jul 1993;143(1):49-64. [Medline].

  32. Pflugfelder SC, Huang AJ, Feuer W, Chuchovski PT, Pereira IC, Tseng SC. Conjunctival cytologic features of primary Sjögren's syndrome. Ophthalmology. Aug 1990;97(8):985-91. [Medline].

  33. Pflugfelder SC, Whitcher JP, Daniels T. Sjogren's syndrome. In: Pepose J, Holland G, Wilhelmin K. Ocular Infection and Immunity. Mosby: St. Louis.

  34. Prause JU, Frost-Larsen K, Hoj L, Isager H, Manthorpe R. Lacrimal and salivary secretion in Sjögren's syndrome: the effect of systemic treatment with bromhexine. Acta Ophthalmol (Copenh). Jun 1984;62(3):489-97. [Medline].

  35. 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].

  36. Shearn MA. Sjogren's Syndrome. Major Problems in Internal Medicine. Vol 2. WB Saunders; 1971.

  37. Sjogren HS. Zur kenntnis der keratoconjunctivitis sicca (kratitis folliforms bei hypojunktion der tramemdrusen). Acta Ophthalmol (Copen). 1933;11:1-151.

  38. Tabbara KF. Clinical diseases of tear film. In: Smolin G, Thoft R, eds. Cornea: Scientific Foundations and Clinical Practice. 2nd ed. Little Brown and Co: 1987:477-483.

  39. Tabbara KF, Ostler HB, Daniels TE, Sylvester RA, Greenspan JS, Talal N. sjögren's syndrome: a correlation between ocular findings and labial salivary gland histology. Trans Am Acad Ophthalmol Otolaryngol. May-Jun 1974;78(3):OP467-78. [Medline].

  40. Tsubota K. The importance of the Schirmer test with nasal stimulation. Am J Ophthalmol. Jan 15 1991;111(1):106-8. [Medline].

  41. Zoukhri D. Effect of inflammation on lacrimal gland function. Exp Eye Res. May 2006;82(5):885-98. [Medline].

Further Reading

Keywords

SS, keratoconjunctivitis sicca, KCS, xerostomia, dry mouth, dry eyes, autoimmune disorder, rheumatoid arthritis, systemic lupus erythematosus

Contributor Information and Disclosures

Author

James V Aquavella, MD, Professor of Ophthalmology, Department of Ophthalmology, University of Rochester School of Medicine, University of Rochester Eye Institute
James V Aquavella, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, American Medical Association, Contact Lens Association of Ophthalmologists, and International College of Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

Zoe R Williams, MD, Staff Physician, Department of Ophthalmology, University of Rochester School of Medicine, Strong Memorial Hospital
Zoe R Williams, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Ophthalmology
Disclosure: Nothing to disclose.

Shobha Boghani, MD, Assistant Professor, Department of Ophthalmology, Strong Memorial Hospital, University of Rochester
Shobha Boghani, MD is a member of the following medical societies: American Academy of Ophthalmology and Contact Lens Association of Ophthalmologists
Disclosure: Nothing to disclose.

Medical Editor

Fernando H Murillo-Lopez, MD, Senior Surgeon, Unidad Privada de Oftalmologia CEMES
Fernando H Murillo-Lopez, MD is a member of the following medical societies: American Academy of Ophthalmology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Christopher J Rapuano, MD, Professor, Department of Ophthalmology, Jefferson Medical College; Co-Chairman of the Cornea Service, Co-Chairman of Refractive Surgery Department, Wills Eye Hospital
Christopher J Rapuano, MD is a member of the following medical societies: American Academy of Ophthalmology, Eye Bank Association of America, Pennsylvania Medical Society, and Philadelphia County Medical Society
Disclosure: Nothing to disclose.

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.