The most common of the connective tissue diseases, or CTD, is often the least understood and comes with the longest delay in diagnosis. Sjogren’s disease remains an enigma for many doctors and dentists. Clinically, it presents with the typical symptoms of dry eyes and dry mouth, but often patients are unaware of the significance of these symptoms, and their presence may be overlooked by the physicians and dentists who treat them. Though patients are often prescribed eye drops for their dry eyes and saliva substitutes for their oral dryness, other aspects are not investigated.
Epidemiology
The incidence of primary Sjogren’s syndrome, or SS, reported in the literature varies from one in 1,000 to more than one in 100 people. The differences can be attributed to the different criteria used to make the diagnosis and the different populations studied in a retrospective fashion. Other than the proposed San Diego criteria, the frequency has been estimated using the frequency of anti-SS-B antibodies in blood donors, yielding a frequency of one in 2,500 consecutive donors. Clearly in clinical practice, women outnumber men as in all CTDs.
Clinical manifestations
Perhaps the most common clinical symptom in SS patients is fatigue, and certainly the most difficult to determine as being because of the disease. As in the other CTDs, fatigue and malaise are the most difficult to treat but often seem to correlate with disease activity. Many patients are labeled as suffering from fibromyalgia syndrome. Because testing has improved so much over the years, previous work-ups may have shown negative ANAs, whereas today most patients will show positive antibodies to either or both SS-A and SS-B antigens. Active disease may be manifested by skin and vaginal dryness. Rashes may also be present at any time during the course of the disease.
Gastrointestinal problems include reflux symptoms and swallowing difficulties, but rarely serositis (peritonitis). Renal manifestations are rare and should raise alarms that the patient has an overlap syndrome or has morphed into lupus. Respiratory symptoms of dryness are common, but one should be alert to the possibility of pleuritis, pericarditis, and more important, interstitial lung disease. Early diagnosis may help prevent progression with appropriate therapy. Raynaud’s is commonly present and should raise questions of a CTD if the patient is older than 25.
Peripheral neuropathies are common, and the more severe forms such as mononeuritis multiplex often result from vasculitis of large vessels and medium-sized nerves and require immediate aggressive treatment.
Enlargement of lymph nodes should prompt a thorough search for B-cell lymphomas, which are found in increasing frequency in SS patients. Often they present in the cervical or salivary glands, or as a more generalized disease.
Laboratory parameters should be checked at the time of diagnosis and according to symptom exacerbations. The common tests such as ESR, CRP, protein electrophoresis, and CBC are often abnormal with active disease and normalize with treatment or quiescent disease.
Most important, dentists remain on the front line of diagnosis and treatment of Sjogren’s patients. Frequent check-ups are recommended to help prevent further decay, eliminate sources of infections, etc. Only with the help of our dental colleagues can we hope to eliminate the 10-year delay in diagnosis that is so frequently seen in Sjogren’s patients and improve their quality of life. ■
By Sylvia Duby, MD, FACR, FRCPC
Dr. Duby practices rheumatology in Wilmette, Ill., and is an assistant professor at Northwestern University in Chicago. Her areas of interest include Sjogren’s syndrome, osteoporosis, rheumatoid arthritis, and psoriatic arthritis. She believes in treating the whole person using both conventional and complementary therapies. Reach her at [email protected].
