Sjogren Syndrome

Sjögren (pronounced like “show-grin”) syndrome is a systemic inflammatory disease affecting primarily the lacrimal and salivary glands. It may exist as a primary disorder (ie, primary Sjögren syndrome) or can be associated with other autoimmune diseases (ie, secondary Sjögren syndrome), such as rheumatoid arthritis, systemic lupus erythematosus, or systemic sclerosis. Sjögren syndrome is a T cell–driven autoimmune disease and is characterized by focal lymphocytic infiltration of the lacrimal and salivary glands. The etiology of Sjögren syndrome, although still ill-defined, is thought to be multifactorial, involving viral, neural, genetic, and environmental factors. Although the disease itself is not life-threatening, the common occurrence of dry mouth in Sjögren syndrome not only impacts quality of life but can lead to complications that increase the risks of morbidity and mortality among older adults, including candidiasis, dental caries, periodontitis, dysphagia, aspiration pneumonia, and other pulmonary infections.

Yet, the severity of Sjögren syndrome and its oral symptoms appear to be relatively unknown and underrecognized in clinical practice.  Annals of Long-Term Care: Clinical Care and Aging (ALTC) had the opportunity to discuss recognition and treatment of Sjögren syndrome with Driss Zoukhri, PhD, Professor, Department of Diagnosis and Health Promotion, and Mabi Singh, DMD, MS, Associate Professor, Department of Oral Pathology, Oral Medicine and Craniofacial Pain, Tufts University School of Dental Medicine, Boston, MA. Part two of this Ask the Expert will delve into a deeper discussion of diagnosing and treating xerostomia associated with multiple etiologies in older adults.

ALTC: How prevalent is Sjögren syndrome?

The prevalence of Sjögren syndrome varies widely depending on the criteria for classification, but it is estimated that between 1 and 3 million people in North American suffer from this disease. According to the Sjögren’s Syndrome Foundation, as many as four million Americans are living with this disease. Since Sjӧgren syndrome is a very underdiagnosed condition, it is difficult to estimate the prevalence. 

Why does Sjögren syndrome present more frequently in postmenopausal women than in other patient populations?

Several studies reported that androgens possess anti-inflammatory properties whereas estrogens were found to be proinflammatory. Androgen deficiency does occur during aging and after menopause. Furthermore, the serum levels of androgen precursors, hormones, and metabolites have been shown to be low in patients with Sjögren syndrome compared to controls. Therefore, postmenopausal women are at higher risk of developing Sjögren’s syndrome.

What are the oral signs and symptoms of Sjögren syndrome?

Subjective sensation of the dryness of mouth (xerostomia), increased thirst, fissured or lobulated and coating on tongue, burning sensation of mouth, difficulty eating, swallowing, and loss of taste are the common symptoms. Sticking of oral tissue especially the lips to the teeth, and the tongue to the roof of mouth, chapped lips, cracks at the corners of mouth and debris on teeth and palate are also seen, as well as increased experience of development of new dental carious lesions and recurrent carious lesions leading to failure of restorations.

Does every patient with Sjögren syndrome experience some degree of dry mouth?

There is no direct linear correlation with the reduction of saliva and the experience of dry mouth. Some researchers have found that there could be a compensatory mechanism in the beginning stages of Sjögren syndrome. The degree of dry mouth is basically the function of extent of damage to the salivary glands and the remaining functional salivary glands. Generally, when the basal unstimulated salivary volume goes down anywhere from 50% to 75% of the original volume, experience of dry mouth occurs. In autoimmune diseases like Sjögren syndrome, the serous (watery) part of the salivary glands are destroyed first, resulting in thick, viscous and mucous saliva, and patients may feel that there is excessive saliva in the mouth.

How is Sjögren syndrome diagnosed?

Sjögren syndrome is considered to be the most commonly underdiagnosed autoimmune disease. It is not uncommon for there to be a delay of 5 to 8 years after symptom onset before a diagnosis is made. The diagnostic approach to Sjögren syndrome is complicated because it must include two different goals: first, assessment of the ocular and salivary components; and second, differentiation between the primary and secondary variants of the syndrome.

Are biopsies necessary to confirm diagnosis?

The markers (circulatory antibodies) for Sjögren syndrome in the blood are anti-Ro (SSA) and anti-La (SSB). These are only about 60% sensitive, meaning 40 % of the times, the result will be shown as negative. The biopsy of the minor salivary glands of the low lip is still the gold standard to diagnose Sjögren syndrome. Harvesting 7 to 10 minor salivary glands and microscopic examination at different levels are necessary for proper diagnosis. Presence of 50 or more lymphocytes in 4 mm² area is considered positive for Sjӧgren’s syndrome.

Some opinion-based articles have stated that many physicians do not take the time to test for Sjögren syndrome in older adults who present with complaints of oral dryness. Why do you think that is?

Sjögren syndrome is a very underrecognized condition. Since many of the older adults are taking one or more medications, the physicians and dentists oftenattribute the condition as a side effect of the medications. That is one of the reasons why the diagnosis takes so long (on average, 8 years) after the complaint of dryness in the mouth. The changes in qualitative and quantitative saliva are not measured in the physician’s or dentist’s office. Rather, the patients are often advised to drink/sip water as a remedy for the subjective sensation of dryness in the mouth. In our clinical practice we find patients who are told that the problem is in their head.

In your experience, have you encountered any atypical presentations of Sjögren syndrome in an older adult?

We have diagnosed Sjögren syndrome on patients who have come for the treatments of common oral lesions (eg, lichen planus) or those who have tooth surface loss due to gastroesophageal reflux disease. Many patients who reported a strange taste and burning sensation in their mouth were diagnosed with Sjögren syndrome. Discomfort in wearing full and partial dentures also have led to the diagnosis of Sjogren syndrome. The fullness and swelling in the parotid and submandibular gland region also lead to the suspicion of Sjӧgren syndrome.  

How is Sjögren syndrome treated?

Treatment is basically directed towards the alleviation of the symptoms, such as dry mouth and dry eyes. Apart from using sialogogues (titrated to maximum dose), to increase the salivary flow, other potential complications of salivary hypofunction, also have to be treated to reduce the risk factors in the oral cavity.

In the second part of this Q&A, the experts discuss treatment of xerostomia associated with Sjögren syndrome and other etiologies. The experts also review the prevalence and burden of xerostomia and its diagnosis in older adults. Access Part 2 here.