Oral Sjogren's Syndrome Manifestations
by James Scuibba, M.D.
- Background
- Introduction
- Clinical Presentation
- Diagnosis/Diagnostic Assays
- Management
- References
- Read more about Sjögen's Syndrome
The oral component of Sjogren's syndrome remains a problematic clinical presentation of this condition and, from the patients perspective, is perhaps the most annoying and difficult of all associated symptoms. The historical development of what we consider Sjogren's syndrome today traces its early origins to that of Hadden, who , in 1871 noted an association between the concurrent presence of dry eye and dry mouth. In 1888 Mickulicz demonstrated the presence of a round cell infiltration into the enlarged parotid and lacrimal glands. In 1925 the French physician Gougerot related dry eyes and dry mouth to an overriding exocrine gland abnormality. Dr. Henrik Sjogren, in 1933, was the first to correlate the dry eye dry mouth condition or so-called keratoconjunctivitis sicca and rheumatoid arthritis. Finally, in 1943 the English physician, Morgan, was the first to equate Sjogren's syndrome and lymphoproliferative diseases.
IntroductionThe oral aspects of primary Sjogren's syndrome consist of mucosal atrophy (80% to 95%), salivary gland enlargement(approximately 30 %),
(CT scan)
recurrent parotitis, reduced salivary flow rate (xerostomia)
and an increased incidence of dental caries.
The oral manifestations therefore of Sjogren's syndrome may include xerostomia with or without salivary gland enlargement, candidiasis, dental caries and taste dysfunction. As a result of Sjogren's syndrome, the major problem within the oral cavity relates to understanding the role of saliva and what its presence as an important biologic fluid subserves. We have chosen to define six major protective functions afforded by the presence of saliva which include:
- coating and lubrication of the oral mucous membranes
- physical debridement or mechanical cleansing of the oral cavity
- maintenance of an essentially neutral pH in the mouth by virtue of its inherent buffer capacity
- maintenance of tooth structure by way of specific components within saliva
- a remineralization and tooth-protective function
- Finally, saliva contains numerous compounds including antibodies and proteins that subserve an antimicrobial function, which includes protection against bacteria, fungi as well as some viruses.
The functions of saliva will be addressed by examining some of its key components and relate the absence of such to an accompanying dysfunction. The key components of saliva related to mucosal lubrication include mucins, proline-rich glycoproteins and water. The dysfunction resulting from absence of saliva therefore would include dryness, mucous membrane friability and altered oral sensation. The absence of water and mucin will hinder formation of an adequate food bolus and therefore ensuing swallowing difficulties or so-called dysphagia. An important aspect of salivary content relates to remineralization secondary to the presence of anionic rich proteins as well as a compound known as statherin. Absence of these agents contributes to a much greater risk of development of dental decay or caries.
Individuals with Sjogren's syndrome often will complain of taste alterations which may in fact be related to decreased solubility of food within the oral cavity by virtue of less fluid available and the associated absence of a substance known as gustin, a normal constituent found in salivary fluid. The antimicrobial aspect of saliva is related to the presence of several key components, namely secretory IgA as well as alpha and beta defensins, lysozymes, lactoperoxidase, lactoferrin, and histidine-rich proteins or so-called histatins. In the absence of saliva, these important elements are missing, therefore, one may expect an increased incidence of intraoral and oropharyngeal yeast infections, gum disease and dental decay.
During the 24 hour daily cycle, salivary flow tends to be rhythmic and uneven in terms of how much saliva is produced at any one time. Peak salivary flow becomes evident in the latter part of the morning and gradually dwindles to its lowest levels during sleep. At rest, the majority of saliva is manufactured by the sublingual and submandibular glands, which are located beneath the tongue, while stimulated saliva is manufactured chiefly by the parotid glands, which are situated on the side of the face in front of the ear and over the posterior mandible.
Diagnostic Assays
(sialogram x-ray)
(sialogram CT scan)
The management or treatment of Sjogren's syndrome is oftentimes empirical, but often relates to use of systemic drugs as well. From the systemic drug treatment standpoint, immunosuppressive therapy in the form of corticosteroids or cytotoxic drugs have proven effective, in particular when symptoms are severe. A drug known as Plaquenil has also proven to be helpful in some cases with open questions remaining as to the role of alpha interferon and nonsteroidal anti-inflammatory drugs.
Specific management strategies for Sjogrens syndrome include maintaining adequate but not excessive levels of hydration coupled with a sensible diet low in carbohydrates and sugars. Frequent dental examinations and practicing of scrupulous oral hygiene will help in retarding dental decay (caries). Use of fluoride rinses, topical fluoride gels and fluoride-containing toothpastes are useful and aid in the remineralization process and a strengthening of the enamel surface making it more resistant to acid dissolution. Artificial saliva, while only modestly effective, can often be used selectively during periods of the day where salivary dysfunction is particularly bothersome. More recently, systemic drugs have become available in the form of pilocarpine and cevimeline which work in a similar manner in stimulating salivary secretion.
A multi-institutional study has demonstrated the efficacy and tolerability of pilocarpine in improving symptoms associated with dry.ref 1 More recently and additional drug, cevimeline, has also shown value in treatment of the dry mouth.ref 2 Both agents are now available for the Sjogrens Syndrome patient suffering from the effects of dry mouth.
Antifungal therapy, both topical and systemic agents, may become necessary for individuals with chronic candidiasis. Oral antimicrobial rinses having chlorhexidene as a major component will reduce dental plaque formation and will reduce oral pathogen loads as well. Finally, use of remineralizing solutions assumes an important role in the management of early dental decay prior to actual cavity formation. Frequent dental visits with a clinician aware of the early carious lesion, coupled with daily at-home dental care including remineralizing agents, such as fluoride and other techniques, will help avoid dental dysfunction and costly restoration procedures.
1. Vivino F, Al-Hashimi I, Khan Z, et al. Arch Int Med 159(2):174, 1999.
2. Fife R, Chase W, Dore D, et al. Arch Int Med 162:1293, 2002.
Additional Resources
Fox RI. Sjögren's Syndrome in Textbook of Rheumatology, Kelley WN, Harris ED, Ruddy S, Sledge, CB. (eds) W.B. Saunders, Co., Philadelphia. pgs. 957-960, 1997.
Additional Resources
Tzioufas AG & Moutsopoulos HM. Sjögren's Syndrome in Rheumatology, Klippel JH & Dieppe PA (eds) Mosby, Philadelphia. pgs. 7.32.5, 1998.
