Sjögren’s Syndrome is a rheumatic autoimmune disease, in
which intense lymphocytic infiltration occurs in the exocrine
glands.2 This causes inflammation, which damages glandular tissue
and impairs function. Primary Sjögren’s Syndrome is diagnosed in
the absence of any other connective-tissue disease, while Secondary
Sjögren’s Syndrome is accompanied by other autoimmune diseases,
such as rheumatoid arthritis, lupus erythematosus, scleroderma, or
in rare cases, Behçet’s disease.3 Secondary Sjögren’s Syndrome com-
prises approximately 60 percent of cases.
Clinical features of Sjögren’s Syndrome
Sjögren’s Syndrome is mainly characterized by dry eyes and a dry mouth, and may also affect other mucosal tissues such as the nose, larynx, gastrointestinal system, and vagina. The systemic auto- immunity associated with Sjögren’s Syndrome can also result in dry skin, fatigue, low-grade fever, constipation, myalgia, and joint pain. Other conditions that might occur include small-vessel vasculitis, Raynaud’s phenomenon, pulmonary symptoms, nephritis, neurop- athy4, thyroiditis, and even lymphoma. Sjögren’s patients have a 16 times greater than normal risk of developing lymphoma.5
Mental impact of Sjögren’s Syndrome
Brain fog is becoming increasingly recognized as a symptom of central nervous system involvement in Sjögren’s Syndrome: it is characterized by mildly impaired short-term memory and con- centration, and slower cognitive processing.6 Little is currently known about the pathogenesis of brain fog, but it is thought that it might involve inflammation of the cerebral small blood ves- sels. In common with many chronic medical diseases, depression
Clinical features of Sjögren’s Syndrome
Sjögren’s Syndrome is mainly characterized by dry eyes and a dry mouth, and may also affect other mucosal tissues such as the nose, larynx, gastrointestinal system, and vagina. The systemic auto- immunity associated with Sjögren’s Syndrome can also result in dry skin, fatigue, low-grade fever, constipation, myalgia, and joint pain. Other conditions that might occur include small-vessel vasculitis, Raynaud’s phenomenon, pulmonary symptoms, nephritis, neurop- athy4, thyroiditis, and even lymphoma. Sjögren’s patients have a 16 times greater than normal risk of developing lymphoma.5
Mental impact of Sjögren’s Syndrome
Brain fog is becoming increasingly recognized as a symptom of central nervous system involvement in Sjögren’s Syndrome: it is characterized by mildly impaired short-term memory and con- centration, and slower cognitive processing.6 Little is currently known about the pathogenesis of brain fog, but it is thought that it might involve inflammation of the cerebral small blood ves- sels. In common with many chronic medical diseases, depression
or anxiety might accompany Sjögren’s Syndrome, related to its
adverse effects on the quality of life. This is shown by its score on
the Devin’s Illness Intrusiveness Scale,7 where its negative impact
on quality of life is comparable with that of multiple sclerosis, or
kidney dialysis.8 Inflammatory cytokines have also been impli-
cated in mood disorders.
Prevalence
Sjögren’s Syndrome is the second-most-common rheumatic autoimmune disease. Approximately four million Americans are affected, and 90 percent of Sjögren’s patients are women.9 Most are diagnosed in their late forties, but the disease can also affect children and men. Men are usually diagnosed later than females.
Diagnosis
Oral Symptoms—a positive response to at least one of the following questions can denote Sjögren’s.
1. Have you had a daily feeling of dry mouth for more than three months?
2. Have you had recurrently or persistently swollen salivary glands as an adult?
3. Do you frequently drink liquids to aid in swallowing dry food?
Ocular Symptoms—a positive response to at least one of the following questions can denote Sjögren’s.
1. Have you had daily, persistent troublesome dry eyes for more than three months?
2. Do you have a recurrent sensation of sand or gravel in the eyes? 3. Do you use tear substitutes more than three times a day?
Prevalence
Sjögren’s Syndrome is the second-most-common rheumatic autoimmune disease. Approximately four million Americans are affected, and 90 percent of Sjögren’s patients are women.9 Most are diagnosed in their late forties, but the disease can also affect children and men. Men are usually diagnosed later than females.
Diagnosis
Oral Symptoms—a positive response to at least one of the following questions can denote Sjögren’s.
1. Have you had a daily feeling of dry mouth for more than three months?
2. Have you had recurrently or persistently swollen salivary glands as an adult?
3. Do you frequently drink liquids to aid in swallowing dry food?
Ocular Symptoms—a positive response to at least one of the following questions can denote Sjögren’s.
1. Have you had daily, persistent troublesome dry eyes for more than three months?
2. Do you have a recurrent sensation of sand or gravel in the eyes? 3. Do you use tear substitutes more than three times a day?
The American College of Rheumatology’s current classifi-
cation criteria for diagnosis of Sjögren’s Sydrome specifies that
patients have oral and ocular symptoms, and meet two of the
following criteria:10
Each objective criterion is allocated points. Anti-SSA positiv- ity, and a positive minor salivary gland biopsy each score three points, while a Schirmer’s test < 5mL/5 min, an ocular staining score >4, and a resting salivary flow < 0.1mL/min each score one point. For a diagnosis of Sjögren’s Syndrome, oral and ocular symptoms must be present, plus a total of four points.
Exclusion criteria
The following conditions, which also cause dry eyes and dry mouth comprise the exclusion criteria; if these are present, then Sjögren’s Syndrome is not diagnosed.
-
Autoantibodies, such as positive blood tests for anti-nuclear
antibody (ANA), plus anti-SSA (Ro), and/or anti-SSB (La),
or positive rheumatoid factor.11
-
Histopathology: Biopsy of the labial minor salivary glands,
which shows focal lymphocytic sialadenitis.
-
Ocular signs: an ocular staining score, such as a rose
Bengal test score of >4, which shows keratoconjunctivitis
sicca, or a Schirmer’s test with a lachrymal flow rate of <
5mm/5 min.
-
Evidence of salivary gland involvement includes a resting
salivary flow rate of < 0.1 ml/ min, parotid sialography
showing dilation of the ducts (sialoectasias), or scintigraphy
that shows low uptake and delayed excretion of an intrave-
nously administered marker.
However, diagnostic criteria are still evolving—at the 13th
Each objective criterion is allocated points. Anti-SSA positiv- ity, and a positive minor salivary gland biopsy each score three points, while a Schirmer’s test < 5mL/5 min, an ocular staining score >4, and a resting salivary flow < 0.1mL/min each score one point. For a diagnosis of Sjögren’s Syndrome, oral and ocular symptoms must be present, plus a total of four points.
Exclusion criteria
The following conditions, which also cause dry eyes and dry mouth comprise the exclusion criteria; if these are present, then Sjögren’s Syndrome is not diagnosed.
-
Pre-existing lymphoma
-
Hepatitis C infection
-
HIV infection
-
Sarcoidosis
-
Duration of anti-cholinergic drug use, which is shorter than
four-fold the drug’s half-life
-
History of head and neck radiation therapy
Assessment of the Sjögren’s patient in the dental office setting
Oral care and support for individuals with Sjögren’s Syndrome begins with thorough assessment, which facilitates timely specialist referral for earlier diagnosis. This includes general appraisal, medical history review, and screening for symptoms using a questionnaire. This should be followed by a
head-and-neck examination, and comprehensive intraoral exam- ination. The Challacombe Scale of Clinical Oral Dryness is also a valuable tool for clinically assessing and quantifying the sever- ity of oral dryness.13 (see pg. 60) This scale is based on a Clinical Oral Dryness Score (CODS), and lists 10 key features of dry mouth; one point is allocated for each feature, and the patient’s additive score indicates the severity of the dry mouth. There is an inverse relationship between salivary flow rates and CODS: high CODS are related to hyposalivation.
Management of a dry mouth needs to be multifaceted to
address the multiple oral symptoms and complications. This
includes stimulation of salivary flow, and conservation of func-
tional salivary gland tissue. Also, saliva substitutes can be used as
required to protect and lubricate, and to facilitate speech, masti-
cation and swallowing. In addition, prevention of complications
is crucial. These include dysphagia and aspiration pneumonia,
sialoliths, salivary gland swelling, caries, periodontal disease,
and soft-tissue infections such as candidiasis. Supplementation
of Omega 3 at 1000mg per day has been shown to improve the
lubricating quality of the saliva and the tears. Application of heat and massage can help to aid salivary flow through the ducts, and
reduce swelling of the parotid and submandibular salivary glands.
Conclusion
In January 2012, the Sjögren’s Syndrome Foundation launched a five-year breakthrough goal, “50 in 5.” The purpose is “to shorten the time to diagnose Sjögren’s by 50 percent in 5 years.”
Increased vigilance and enhanced interdisciplinary collabora- tion between the various health-care professions could facilitate
In January 2012, the Sjögren’s Syndrome Foundation launched a five-year breakthrough goal, “50 in 5.” The purpose is “to shorten the time to diagnose Sjögren’s by 50 percent in 5 years.”
Increased vigilance and enhanced interdisciplinary collabora- tion between the various health-care professions could facilitate
earlier diagnosis, leading to improved outcomes for the Sjögren’s
patient. For example: medical and dental professionals could
include questions on ocular, oral and systemic symptoms of
Sjögren’s Syndrome in their screening questionnaires.
Public awareness of Sjögren’s Syndrome could be raised with more information in the mass media, and increased availability of screening questionnaires for self-assessment. This could enhance patients’ ability to advocate for themselves as they navigate the health-care system. ■
Public awareness of Sjögren’s Syndrome could be raised with more information in the mass media, and increased availability of screening questionnaires for self-assessment. This could enhance patients’ ability to advocate for themselves as they navigate the health-care system. ■
