AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Background

Sjögren syndrome is a chronic autoimmune disorder characterized by xerostomia (dry mouth), xerophthalmia (dry eyes), and lymphocytic infiltration of the exocrine glands. This triad is also known as the sicca complex.

Sjögren syndrome displays a wide spectrum of severity and can go undiagnosed for several months to years. The Swedish physician Henrik Sjögren first described the syndrome in 1933.

For a CME activity, see Systemic Lupus Erythematosus and Sjögren's Syndrome: Highlights From ACR 2007. For more information, see Medscape's Sjögren's Syndrome Resource Center.

Pathophysiology

Sjögren syndrome can occur as a primary disease of exocrine gland dysfunction or in association with several other autoimmune diseases (eg, systemic lupus erythematosus [SLE], rheumatoid arthritis, scleroderma, systemic sclerosis, cryoglobulinemia, polyarteritis nodosa). These primary and secondary types occur with similar frequency, but the sicca complex seems to cause more severe symptoms in the primary form.

Virtually all organs may be involved. The disease commonly affects the eyes, mouth, parotid gland, lungs, kidneys, skin, and nervous system.

Frequency

United States

Sjögren syndrome is estimated to be the second most common rheumatologic disorder, behind only SLE.

International

Comparative studies between different ethnic groups have suggested that Sjögren syndrome is a homogeneous disease that occurs worldwide with similar prevalence and affects 1-2 million people.

Mortality/Morbidity

• Although the disease process rarely shortens a patient's lifespan, morbidity can be devastating. Ocular dryness can lead to chronic keratoconjunctivitis and corneal ulcers. Oral dryness leads to caries, fissures, candidal infections, and difficulty speaking and swallowing food. These patients often are undiagnosed or misdiagnosed for years. They often feel clinicians are uneducated or unsympathetic to their disease.
• As a result of the lymphocytic infiltration, 10% of patients may develop pseudolymphoma, a lymphoproliferative process. Approximately 10% of these patients can develop non-Hodgkin lymphoma (1% of all patients with Sjögren syndrome).

Sex

The female-to-male ratio of Sjögren syndrome is 9:1.

Age

Peak incidence occurs in the fourth and fifth decades of life.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

History

Onset of Sjögren syndrome is insidious, and symptoms may be mild.

• Dry eyes and/or mouth (required for the clinical diagnosis)
• Musculoskeletal (60%)
• • Fatigue (60%)
  • Arthralgias (60-70%)
  • Myalgias (20-30%)
• Dry skin (40%)
• Vaginal dryness (40%)

Physical

The Sjögren syndrome may be mild or hard to detect clinically.

• Keratoconjunctivitis
• Salivary insufficiency
• Vasculitis (25%)
• Lymphadenopathy (20%)
• Polyneuropathies (10-15%)
• Renal involvement
• • Glomerulonephritis
  • Interstitial nephritis
• Parotid gland enlargement (usually bilateral)

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Other Problems to be Considered

Neuropathies
Medication side effects (dry mouth especially)

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Lab Studies

• Schirmer test
• • The Schirmer test is probably the only test available in the ED to strongly support or refute suspicion of Sjögren syndrome.
  • A test strip of number 41 Whatman filter paper is placed near the lower conjunctival sac to measure tear formation.
  • Healthy persons wet 15 mm or more after 5 minutes. A positive test occurs when less than 5 mm is wet after 5 minutes.
• Erythrocyte sedimentation rate (ESR) is elevated in 80% of patients.
• Rheumatoid factor is present in 52% of cases of primary-type Sjögren syndrome and in 98% of secondary-type cases.
• Complete blood count (CBC)
• • A mild normochromic normocytic anemia is present in 50% of patients.
  • Leukopenia occurs in up to 42% of patients.
• Autoantibodies (outside of ED)
• • Anti-SS-A and anti-SS-B are present in most cases of primary-type Sjögren syndrome, while antisalivary duct antibodies are present in most cases of the secondary type.
  • Antinuclear antibodies of the speckled and homogeneous type are present in most cases of primary Sjögren syndrome.
• Creatinine clearance may be diminished in up to 50% of patients.

Imaging Studies

• No imaging studies are helpful in the initial diagnosis of Sjögren syndrome. However, CT scan could be helpful if progression to lymphoma is suspected. This may not be necessary in the emergent setting. MRI of the salivary glands can also be performed outside the ED setting for intraglandular and extraglandular duct dilatations and duct strictures that can be seen with chronic sialadenitis.

Other Tests

• Rose bengal staining is a test performed on an outpatient basis to confirm ocular involvement.

Procedures

• Minor salivary gland or lower lip biopsy
• • For histopathologic confirmation of exocrine gland infiltration
  • Outside the scope of the ED

TREATMENT

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Emergency Department Care

• Diagnosis of Sjögren syndrome can be made from the ED if the index of suspicion is high.
• Patients may present with mild symptoms (eg, eye grittiness, eye dryness or discomfort, dry mouth, recurrent caries). Bilateral parotid gland swelling is also a common presentation.
• Patients with known Sjögren syndrome should not be taken lightly for their complaint of dry eyes or dry mouth, as these chronic problems can be very distressing and obtrusive.

Consultations

• Surgical consultation for lip biopsy as an outpatient is needed to histologically confirm the diagnosis.
• All patients with known or suspected Sjögren syndrome should be referred to a rheumatologist.

MEDICATION

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Aids to lubrication are the mainstay of therapy for the ocular and oral complications of Sjögren syndrome. No cure for this disease exists.

Drug Category: Lubricants

These agents may be needed every 1-3 h. Thicker, longer-acting drops are available, but they may cause blurring of vision due to the film produced on the cornea and are more appropriate for bedtime use.

Drug Category: Salivary aids

Use of sugarless chewing gums and salivary substitutes assists in management of oral symptoms.

Drug Category: Cholinergic agents

These agents increase activity of exocrine glands, including salivary glands.

Drug Category: Immunosuppressive agents

These agents are used with varying degrees of success for glomerulonephritis, interstitial pneumonitis, and pseudolymphoma. They do not appear to aid in the oral or ocular manifestations.

FOLLOW-UP

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Further Inpatient Care

• Give attention to artificial lubricants and humidified oxygen to intubated and/or sedated patients with Sjögren syndrome.

Further Outpatient Care

• Patients should be encouraged to avoid activities that increase the dryness symptoms (eg, smoking, low-humidity environments).
• All patients with Sjögren syndrome should be monitored by an ophthalmologist and dentist, in addition to their rheumatologist.
• Certain patients may be candidates for punctal occlusion, usually performed by an ophthalmologist.
• The Sjögren Syndrome Foundation, Incorporated, was founded in 1983 and is a good resource for patients. Contact the foundation at 6707 Democracy Blvd, Ste 325, Bethesda, MD, 20817; (800) 475-6473, (301) 530-4415 (fax).

Complications

• The sicca complex of Sjögren syndrome rarely leads to serious complications, but potential problems with other associated rheumatic diseases must be considered.
• A very small risk (1%) of progression to non-Hodgkin lymphoma does exist.

Prognosis

• Prognosis of Sjögren syndrome is good from the standpoint of mortality, but it has significant morbidity.
• Patients can be reassured that the Sjögren syndrome itself will not shorten their lifespan.

Patient Education

• For excellent patient education resources, visit eMedicine's Arthritis Center. Also, see eMedicine's patient education article Sjögren Syndrome.

MISCELLANEOUS

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical/Legal Pitfalls

• Medical pitfalls include failure to recognize Sjögren syndrome. Many times, the symptoms of dry or itchy eyes may be attributed to conjunctivitis or allergic symptoms. Prompt referral from the ED may assist in making a positive diagnosis much sooner.

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

• Anaya J, Talal N. Sjögren's syndrome and connective tissue diseases associated with other immunologic disorders. In: WJ Koopman, ed. Arthritis and Allied Conditions: A Textbook of Rheumatology. 13^th ed. Baltimore: Williams & Wilkins; 1997:1561-80.
• Isenberg DA. Systemic lupus erythematosus and Sjögren's syndrome: historical perspective and ongoing concerns. Arthritis Rheum. Mar 2004;50(3):681-3. [Medline].
• Lane HC, Fauci AS. Sjögren's syndrome. In: Harrison's Principles of Internal Medicine. 11^th ed. New York: McGraw-Hill; 1987:1433-34.
• Ramos-Casals M, Brito-Zeron P, Garcia-Carrasco M, Font J. Sarcoidosis or Sjogren syndrome? Clues to defining mimicry or coexistence in 59 cases. Medicine (Baltimore). Mar 2004;83(2):85-95. [Medline].
• Talal N. Sjögren's syndrome: historical overview and clinical spectrum of disease. Rheum Dis Clin North Am. Aug 1992;18(3):507-15. [Medline].
• Wallach J. Interpretation of Diagnostic Tests: A Synopsis of Laboratory Medicine. 4^th ed. Boston: Little, Brown; 1986:321-3.

Article Last Updated: Aug 19, 2008