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American Pain Society
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Robert J. Gatchel, PhD, Department Editor
Kate McGraw, MHS; H. Dwight Cavanagh, MD PhD
Sjögren's syndrome (SS) is an inflammatory autoimmune disorder of the exocrine system that is chronic and slowly progressive. It may develop from single-organ involvement to a widespread systemic pathology that may affect the lungs, kidneys, muscles, central and peripheral nervous systems, and blood vessels, and may disturb processes at the cellular level. This painful, debilitating disorder is more common in women, affecting up to 3% of all women over the age of 55 (Price & Venables, 1995).
Although infrequently diagnosed in men, SS produces serological and clinical characteristics similar to those seen in women (Anaya et al., 1995).
The disorder, like many diseases, appears to result from an interplay between various environmental and predispositional factors, with no known heritability. Current research focuses on retrovirus exposure as a possible etiological factor (Isenberg, 1995). However, no single environmental factor is necessary or sufficient for the development of the disorder (Price & Venables, 1995).
The symptoms of SS can be primary (i.e., occur in the absence of other significant disease) or may occur as a result of autonomic neuropathy from a preexisting condition such as diabetes, thyroid disease, or systemic lupus erythematosus (Perez, Kraus, Lopez, Cifuentes, & Alarcon-Segovia, 1995; Schumacher, 1993; Utset et al., 1994). The course of the disorder is unremitting, irreversible, and progressive, with complications leading to premature death in some instances.
The main symptoms of SS are excessive dryness of the mouth and eyes, apparently caused by lymphocytic infiltration of the exocrine glands. Other symptoms include difficulty swallowing or chewing due to lack of saliva, and an accompanying increase in dental caries. Clinical presentation varies greatly from patient to patient because of the unpredictable course of the disease and the numerous variations of organ and systemic involvement (see Table 1). For instance, central nervous system involvement may mimic the symptoms of multiple sclerosis. Gastrointestinal manifestations may involve epigastric pain and nausea (Sheikh & Shaw-Stiffel, 1995). The wide variation in the clinical presentation of SS contributes to the challenge of determining an accurate, timely diagnosis. The nature of the pain varies from patient to patient, based on the specific SS manifestation. The pain can range from a gritty sensation in the eyes to more widespread discomfort due to joint or muscle involvement, as well as inflammation.
| CLINICAL MANIFESTATION | PERCENTAGE | |
|---|---|---|
| Arthralgias/Arthritis | 60% | |
| Raynaud's phenomenon | 37% | |
| Lymphadenopathy | 14% | |
| Lung involvement | 14% | |
| Vasculitis | 11% | |
| Kidney involvement | 9% | |
| Liver involvement | 6% | |
| Lymphoma | 6% | |
| Splenomegaly | 3% | |
| Peripheral neuropathy | 5% | |
| Myositis | 1% | |
| Source: Schumacher (1993) | ||
Diagnostic criteria developed in 1993 (see the list below) include ocular symptoms (dry eyes daily for more than 3 months, recurrent sandy sensation in the eyes, or use of tear substitutes more than three times daily), and oral symptoms (feeling of dry mouth more than 3 months, recurrent or persistently swollen salivary glands as an adult, or frequent drinking of liquids to aid in swallowing). In addition, patients may present with positive ocular signs (either a positive Schirmer-1 test or rose bengal score), salivary gland involvement (revealed through salivary scintigraphy, parotid sialography, or unstimulated salivary flow), positive histopathological findings, and the presence of either SS-A, SS-B antigens, antinuclear antibodies, or rheumatoid factor in the serum (Schumacher, 1993). As research has progressed, other criteria, such as impaired sweating (Katayma et al., 1995) have emerged. Newer techniques, such as magnetic resonance imaging (MRI) of the parotid gland, may prove to be valuable noninvasive methods of diagnosing SS (Izumi et al., 1996).
The preliminary criteria for the classification of SS (Vitali, Bomberdieri, & Moutsopoulos, 1993) are as follows (probable SS = three of six criteria are met; definite = four of six criteria are met):
Currently, there remains some confusion as to the precise definition of SS. Thus, diagnosticians may have difficulty reaching a diagnosis and instituting timely, appropriate therapy (Fox, 1992). The number of patients diagnosed with this syndrome appears to be increasing, but this increase may be due to the recent improvement in diagnostic techniques. Most SS patients are diagnosed through a team effort involving the primary care provider, rheumatologist, dentist, and ophthalmologist. Together, this team develops a specific treatment plan for the individual patient. A need exists for a multidisciplinary approach to best manage these patients.
Because there is no cure for SS, medical treatment is primarily palliative. No current treatment completely eliminates the symptoms of the disorder. Most SS patients receive conservative therapy involving tear replacement and intensive oral hygiene. In addition, a saliva substitute is used to alleviate dry mouth sensations. Not all patients experience all therapies with the same level of satisfaction. Recent studies indicate that individual patient response to saliva substitutes currently on the market depends on the patient's saliva flow rate. Often, a patient must experiment with a number of substitutes that have a range of viscoelastic properties before finding a substitute that is subjectively efficacious ('s-Gravenmade & Vissink, 1992). Other therapies, depending on individual differences in systemic involvement of SS, are often helpful in controlling or alleviating the course of progression of SS. Some research is currently investigating the effects of different pharmacological interventions on the disease process itself. One recent study indicated that cyclosporine, administered daily early in life, successfully reduced ocular and lacrimal gland disease in genetically isolated mice to produce autoimmune dysfunction similar to that of SS (Jabs, Lee, Burek, Saboori, & Prendergast, 1996). Although these results seem promising, scientifically valid conclusions about the efficacy of this intervention will require replications with humans.
Cognitive and neuropsychiatric symptoms in primary SS include the spectrum of organic brain dysfunction, and are treatable in some cases (Ohtsuka et al., 1995). For instance, a 48-year-old man with primary SS developed subcortical dementia, manifested by memory disturbance, poor concentration and attention, slowed cognition, and sudden personality changes. His condition improved following corticosteroid treatment (Kawashima, Shindo, & Kohn, 1993). This case illustrates the need for continuous monitoring of the mental status of SS patients for any changes that would indicate central nervous system involvement (De Backer & Dehaene, 1995).
When a disease is slowly progressive and debilitating, patients must adjust constantly to cope with the difficulties in functioning that result. This stresses relationships within families, often damaging them irreparably. Consequently, depression and anxiety are the most common psychiatric conditions that coexist with SS (Sagawa, Tanimura, Shinohara, & Warabi, 1995; Utset et al., 1994). Whether the depression or anxiety is intrinsic to the illness or is a manifestation of the disease is unclear and warrants further research. Other issues for research include patients' coping strategies, the role of social support, and the use of psychopharmacological or psychotherapeutic interventions. Obviously, managing the patient's complex array of symptoms and providing preventive and maintenance interventions call for a multidisciplinary treatment approach. At the University of Texas Southwestern Medical Center at Dallas, a multidisciplinary treatment team is being developed at the Southwestern Regional Center for Sjögren's Syndrome. The team, which includes an ophthalmologist, a dentist, a rheumatologist, an internist, and a clinical psychologist, will provide comprehensive management strategies to combat the effects of this disease.
SS is a rare disorder that creates much pain and discomfort. Further research efforts will improve understanding and identify factors amenable to intervention, thus helping healthcare providers alleviate, at least in part, the attendant suffering. In response to this need for further research, the National Institutes of Health (NIH) has issued a Research Funding Application for Developmental Grants for the Establishment of Comprehensive, Oral, Health, and Research Centers of Discovery, as SS is one of the oral health problems that qualifies for NIH funding. As emphasized, a diversified treatment team comprised of appropriate professionals who are able to offer the most inclusive, individualized treatment for SS patients can contribute significantly to advancements in successful current interventions and guide future research.
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De Backer, H., & Dehaene, I. (1995). Central nervous system disease in primary Sjögren's syndrome. Acta Neurologica Belgica, 95(3), 142-146.
Fox, R. (1992). Treatment of the patient with Sjögren's syndrome. Rheumatic Diseases Clinics of North America, 18, 699-709.
Isenberg, D. (1995). Sjögren's syndrome—a house of cards. Clinical Rheumatology, 14 Suppl. 1, 8-10.
Izumi, N., Eguchi, K., Ohki, M., Uetani, M., Hyashi, K., Kita, M., Nagataki, S., & Nakamura, T. (1996). MR imaging of the parotid gland in Sjögren's syndrome: A proposal for new diagnostic criteria. American Journal of Roentgenology, 166, 1483-1487.
Jabs, D., Lee, B., Burek, C., Saboori, A., & Prendergast, R. (1996). Cyclosporine therapy suppresses ocular and lacrimal gland disease in MRL/Mp-lpr/lpr mice. Investigative Opthalmology & Visual Science, 37(2), 377-383.
Katayama, I., Yokozeki, H., & Nishioka, K. (1995). Impaired sweating as an exocrine manifestation in Sjögren's syndrome. British Journal of Dermatology, 133(5), 716-720.
Kawashima, N., Shindo, R., & Kohn, M. (1993). Primary Sjögren's syndrome with subcortical dementia. Internal Medicine, 32, 561-564.
Ohtsuka, T., Saiko, Y., Hasegawa, M., Tatsuno, M., Takita, S., Arita, M., & Okuyama, K. (1995). Central nervous system disease in a child with primary Sjögren syndrome. Journal of Pediatrics, 127, 961-963.
Perez, B., Kraus, A., Lopez, G., Cifuentes, M., & Alarcon-Segovia, D. (1995). Autoimmune thyroid disease in primary Sjögren's syndrome. American Journal of Medicine, 99, 480-484.
Price, E., & Venables, P. (1995). The etiopathogenesis of Sjögren's syndrome. Seminars in Arthritis & Rheumatism, 25(2), 117-133.
's-Gravenmade, E., & Vissink, A. (1992). Management of the oral features of Sjögren's syndrome. Netherlands Journal of Medicine, 40(304), 117-124.
Sagawa, A., Tanimura, K., Shinohara, M., & Warabi, T. (1995). Neuro-psychiatric involvement in primary Sjögren's syndrome. Nippon Rinsho-Japanese Journal of Clinical Medicine, 53, 2563-2567.
Schumacher, H. (Ed.). (1993). Primer on the rheumatic diseases. Atlanta: Arthritis Foundation.
Sheikh, S., & Shaw-Stiffel, T. (1995). The gastrointestinal manifestations of Sjögren's syndrome. American Journal of Gastroenterology, 90(1), 9-14.
Utset, T., Golden, M., Siberry, G., Kiri, N., Crum, R., & Petri, M. (1994). Depressive symptoms in patients with systemic lupus erythematosus: Association with central nervous system lupus and Sjögren's syndrome. Journal of Rheumatology, 21, 2039-2045.
Vitali, C., Bomberdieri, S., & Moutsopoulos, H. (1993). Preliminary criteria for the classification of Sjögren's syndrome. Arthritis Rheumatology, 36, 340-347.
Kate McGraw is a doctoral candidate and clinical psychology intern at the University of Texas Southwestern Medical Center at Dallas; H. Dwight Cavanagh is professor and vice chair of opthalmology at the University of Texas Southwestern Medical Center at Dallas.
