Sjögren Disease

Key Points

  • Sjögren disease is an autoimmune disease that can result in the destruction of exocrine glandular epithelium.
  • The most common symptoms are dry mouth and dry eyes.
  • Specific oral manifestations associated with Sjögren disease may include increased risk of caries, gingivitis, oral candidiasis, enlarged salivary glands, and others.
  • Treatment of Sjögren disease is primarily supportive/palliative, focusing on symptom relief and prevention.
Introduction
Sjögren disease (formerly known as Sjögren’s syndrome) is a chronic, systemic autoimmune disease primarily affecting the salivary glands in the oral cavity and the lacrimal glands in the eyes.1-3 An estimated 3 million people in the U.S. have Sjögren disease and it occurs more often in women (86%) than in men2, 4, 5 with peak incidence occurring around age 50 years.6 Primary Sjögren disease is diagnosed when it is the only autoimmune disease present and secondary (or “associated”) Sjögren disease is diagnosed when there is at least one other concomitant autoimmune disease (e.g., rheumatoid arthritis, systemic lupus erythematosus, or dermatomyositis).1-3, 5

Disease Description

Causes. Sjögren disease is a result of autoimmune destruction of exocrine glandular epithelium, affecting the salivary and lacrimal glands in particular.3 The development of Sjögren disease is influenced by both genetic and environmental factors, and risk factors for disease may include genetic predisposition, family history of Sjögren or other autoimmune disease, or infection with cytomegalovirus or Epstein-Barr virus.3, 7

Symptoms. Although Sjögren disease is a systemic condition that can affect any body organ or system, the primary symptoms may present as Sicca syndrome, which consists of dry eyes (keraconjunctivits sicca) and dry mouth (xerostomia) due to impaired functioning of the lacrimal and salivary glands.1, 3 In a cohort from the NIDCR/NIH Sjögren's Syndrome Clinic, 87.4% of people with confirmed Sjögren disease reported dry mouth.8 Other organ systems can be affected as well (e.g., nasal passages, vagina, skin).3 Skin involvement is relatively common, and may manifest as xeroderma (dry skin), eyelid dermatitis, annular erythema, or cutaneous vasculitis.3 Dermatologic manifestations are frequently complicated by symptoms of itching and burning, along with severely dry skin and hair.3 Other extraglandular symptoms may include fatigue, joint pain, arthritis, Reynaud’s phenomenon, autoimmune thyroiditis, sleep disturbances, anxiety, and depression.3, 9

Diagnosis. Diagnosis of Sjögren disease can be challenging, as symptoms of dry eye/mouth and fatigue can be associated with other more common conditions (e.g., fibromyalgia)5 as well as from medications.9 Formal classification criteria adopted by the American College of Rheumatology/European League Against Rheumatism10 require the presence of immunologic abnormalities (i.e., the presence of serum anti-Sjögren’s syndrome–related antigen A [SSA] antibodies or focal lymphocytic sialadenitis seen on labial salivary gland biopsy).5 Ultrasonography of the parotid and submandibular salivary glands may reveal hypoechoic or anechoic areas in patients with the disease.11 Although this method of evaluation is not included in the formal diagnostic classification criteria, salivary gland ultrasound may be a useful tool in diagnosis or monitoring the condition over time.5, 11

Salivary flow measurement may also be considered to assess the baseline salivary gland function. Objective measures of reduced salivary flow include unstimulated salivary flow of 0.1 mL/min or less (measured for 5 to 15 minutes) or chewing-stimulated salivary flow of 0.7 mL/min or less (measured for 5 minutes).12, 13

Complications. People with Sjögren disease may also have mild anemia, increased serum immune globulin and erythrocyte sedimentation rate, positive rheumatoid factor, joint pain (from other musculoskeletal autoimmune diseases), and neurologic issues.2, 5 If left undiagnosed, untreated organ involvement can lead to organ failure and death.9 Additionally, the risk of B-cell lymphoma is increased by 15- to 20-fold among patients with primary Sjögren disease, as compared with the general population.5

Treatment. Treatment for Sjögren disease is primarily supportive/palliative, as there is no cure.2 Alcohol, smoking, and environmental factors such as wind and low humidity can be avoided as they may exacerbate Sicca symptoms.9 Sialagogues (i.e., salivary stimulants) such as the muscarinic agonists pilocarpine hydrochloride and cevimeline hydrochloride can improve symptoms of dry mouth; however, these agents may cause excessive sweating. This adverse effect may be reduced by gradually increasing dose over time.5 Dry eyes are often treated with preservative-free teardrops and lubricating ointments. Therapy for systemic involvement is tailored towards the specific organ that is affected.9

A 2019 meta-analysis by Chu et al. looking at treatment for primary Sjögren disease found that reducing inflammation may potentially improve salivary gland function, no immunomodulatory drugs provide consistent benefit for dry mouth and dry eye symptoms, and that further studies are needed to determine which disease outcomes are potentially sensitive to interventions.14

Oral Manifestations

Because of the hallmark oral manifestations of Sjögren disease, dental professionals are often the first clinicians to detect the condition.2

Due to the xerostomia symptoms that commonly accompany Sjögren disease, patients may present with dry and friable oral mucosa with a dry and fissured tongue.12, 15, 16 Patients with Sjögren disease may commonly have root, cervical, or incisal/cuspal tip dental caries, accumulation of plaque, gingivitis, and/or periodontitis.12, 13, 15, 16

Sjögren disease may also be associated with the following oral health complications:12, 13, 15, 16

  • Oral infections such as candidiasis
  • Enlargement of salivary glands
  • Angular cheilitis
  • Mucositis
  • Traumatic oral lesions
  • Difficulty wearing or retaining oral prostheses

There is some question as to whether the increased incidence of caries in patients with Sjögren disease is due solely to decreased salivary flow rate, or if there are changes in the components of saliva that contribute to increased risk of caries. In a cross-sectional study by Berman et al.17, incidence of caries was compared in participants with salivary hypofunction who either did or did not have a formal diagnosis of Sjögren disease. Results of the study indicated that the participants with Sjögren disease had significantly more caries than did the participants who did not have Sjögren disease, with no differences in salivary flow rate between the groups. Additionally, among the Sjögren disease participants, there was no significant correlation between salivary flow rate and number of caries, suggesting possible qualitative rather than quantitative differences in saliva in individuals with Sjögren disease.17

Dental Patient Management

In patients without a formal diagnosis of Sjögren disease, a thorough head and neck examination, including an intraoral evaluation to identify the presence or absence of salivary pooling on the floor of the mouth, can help a dentist identify who might benefit from further diagnostic evaluations, such as salivary flow rate measurement, minor salivary gland biopsy, or blood and microbial tests.15, 16

A 2018 case report18 published in JADA describes the dental management of a patient with secondary Sjögren disease (in conjunction with systemic lupus erythematosus and rheumatoid arthritis). The patient presented with hyposalivation, rampant caries, and extreme dental sensitivity, which made it difficult to achieve optimal oral hygiene. Following periodontal debridement, the patient’s caries was managed in a minimally invasive manner, using repeat applications of silver diamine fluoride, fluoride varnish application, partial caries removal, and placement of glass-ionomer cement restorations. Because of limited mobility due to rheumatoid arthritis, the oral hygiene regimen was improved with the use of a powered toothbrush, floss holders, and an oral irrigation device, along with mouthrinses. The patient also received education about diet, including limiting intake of fermentable carbohydrates and switching from honey-sweetened beverages to plain water or beverages with artificial sweeteners.

Since patients with Sjögren disease may have difficulty wearing dentures due to dry and sensitive oral mucosa, dental implants may be another option for missing teeth. A 2019 systematic review by Chrcanovic et al. found that the failure rate of dental implants in patients with Sjögren disease is 4.1%, with mean removal time of 12.9 months following placement.19

In 2016, evidence-based guidelines from the Sjögren’s Syndrome Foundation20 came to the following recommendations regarding oral management of patients with Sjögren disease with dry mouth (Table).

Table. Sjögren Syndrome Foundation Clinical Guideline: Oral Health Management Recommendations for Patients with Sjögren Disease with Dry Mouth20

Recommendation
Strength of Recommendation
Topical fluoride should be used in all patients
Strong
Although no study results link improved salivary
flow to caries prevention, the oral health community
generally accepts that increasing saliva may
contribute to decreased caries incidence,
so increasing saliva through gustatory, masticatory,
or pharmaceutical stimulation may be considered
Weak
Chlorhexidine administered as varnish, gel, or rinse may be considered
Weak
Nonfluoride remineralizing agents may be considered as an adjunct therapy
Moderate

Adapted from Zero et al. 201621

For information on the management of dry mouth symptoms that may accompany Sjögren disease, visit the ADA Oral Health Topics page on xerostomia.

References
  1. Melko K. The Daily Struggle with Sjogren's Syndrome: Guiding Dental Patients to Relief from Pain. RDH July 2017. Accessed October 19, 2021.
  2. Cartee DL, Maker S, Dalonges D, Manski MC. Sjogren's Syndrome: Oral Manifestations and Treatment, a Dental Perspective. J Dent Hyg 2015;89(6):365-71.
  3. Generali E, Costanzo A, Mainetti C, Selmi C. Cutaneous and Mucosal Manifestations of Sjogren's Syndrome. Clin Rev Allergy Immunol 2017;53(3):357-70.
  4. Maciel G, Crowson CS, Matteson EL, Cornec D. Prevalence of Primary Sjogren's Syndrome in a US Population-Based Cohort. Arthritis Care Res (Hoboken) 2017;69(10):1612-16.
  5. Mariette X, Criswell LA. Primary Sjogren's Syndrome. N Engl J Med 2018;378(10):931-39.
  6. Qin B, Wang J, Yang Z, et al. Epidemiology of primary Sjogren's syndrome: a systematic review and meta-analysis. Ann Rheum Dis 2015;74(11):1983-9.
  7. Igoe A, Scofield RH. Autoimmunity and infection in Sjogren's syndrome. Curr Opin Rheumatol 2013;25(4):480-7.
  8. Billings M, Dye BA, Iafolla T, et al. Significance and Implications of Patient-reported Xerostomia in Sjogren's Syndrome: Findings From the National Institutes of Health Cohort. EBioMedicine 2016;12:270-79.
  9. Ramos-Casals M, Brito-Zeron P, Siso-Almirall A, Bosch X. Primary Sjogren syndrome. Bmj 2012;344:e3821.
  10. Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjogren's Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts. Arthritis Rheumatol 2017;69(1):35-45.
  11. Hammenfors DS, Brun JG, Jonsson R, Jonsson MV. Diagnostic utility of major salivary gland ultrasonography in primary Sjogren's syndrome. Clin Exp Rheumatol 2015;33(1):56-62.
  12. Navazesh M, Kumar SK. Xerostomia: prevalence, diagnosis, and management. Compend Contin Educ Dent 2009;30(6):326-8, 31-2; quiz 33-4.
  13. Fox PC, Ship JA. Salivary gland diseases. Burket's Oral Medicine, Diagnosis & Treatment: People's Medical Publishing House USA Ltd (PMPH); 2008. p. 191-222.
  14. Chu LL, Cui K, Pope JE. A Meta-Analysis of Treatment for Primary Sjogren's Syndrome. Arthritis Care Res (Hoboken) 2019.
  15. Plemons JM, Al-Hashimi I, Marek CL. Managing xerostomia and salivary gland hypofunction: executive summary of a report from the American Dental Association Council on Scientific Affairs. J Am Dent Assoc 2014;145(8):867-73.
  16. Turner MD, Ship JA. Dry mouth and its effects on the oral health of elderly people. J Am Dent Assoc 2007;138 Suppl:15S-20S.
  17. Berman N, Vivino F, Baker J, Dunham J, Pinto A. Risk factors for caries development in Primary Sjogren's Syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol 2019;In Press.
  18. Young DA, Frostad-Thomas A, Gold J, Wong A. Secondary Sjogren syndrome: A case report using silver diamine fluoride and glass ionomer cement. J Am Dent Assoc 2018;149(8):731-41.
  19. Chrcanovic BR, Kisch J, Wennerberg A. Dental implants in patients with Sjogren's syndrome: a case series and a systematic review. Int J Oral Maxillofac Surg 2019.
  20. Zero DT, Brennan MT, Daniels TE, et al. Clinical practice guidelines for oral management of Sjogren disease: Dental caries prevention. J Am Dent Assoc 2016;147(4):295-305.

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Last Updated: October 19, 2021

Prepared by:

Department of Scientific Information, Evidence Synthesis & Translation Research, ADA Science & Research Institute, LLC.


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