Strides in Diagnosing, Treating Sjögren’s
“One interesting change is the prevalence of Sjögren’s,” said Frederick B. Vivino, MD, a rheumatologist at Penn Rheumatology Associates and the Sjögren’s Syndrome Center in Philadelphia. “Many experts consider it to be the second most common autoimmune rheumatic disease.” In his presentation, “Sjögren’s Syndrome: Comprehensive Diagnosis and Management,” he stressed that, “just like in lupus, you’re dealing with an entire spectrum of disease.” Dr. Vivino reviewed diagnosis of Sjögren’s, including unusual presentations of symptoms like inflammatory myositis, fever of unknown origin, chronic fatigue syndrome, elevated erythrocyte sedimentation rate, and leukocytoclastic vasculitis.
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“Sicca symptoms may be minimal or nil,” warned Dr. Vivino. There may be a discrepancy between symptoms and results of objective tests of eye and mouth dryness. “The bottom line is that if you have a patient with symptoms that fit, you’re obligated to order tests for Sjögren’s.”
He stressed using the American-European criteria for earlier and better diagnosis of Sjögren’s syndrome. “These criteria are in use in nearly every children’s center in the world,” said Dr. Vivino. “They clearly represent an advance over what we had before.”
The criteria call for four of the following six symptoms to be present for diagnosis:
- Dry eye symptoms; and
- Dry mouth symptoms.
- Abnormal test for dry eyes;
- Abnormal test for dry mouth;
- Positive anti-SSA/SSB*; or
- Positive lip biopsy.*
“One of the four objective criteria must be proof of the presence of autoantibodies,” Dr. Vivino pointed out. “This clearly represents an advance over what we had before.”
Errors are often made in reading salivary gland biopsies; a previous study by Dr. Vivino found that 53% of 60 accessions had errors. Other problems include lack of focus score, misinterpretation of focus score, and failure to examine all sections. “It behooves us to talk to our pathologists about accurate test results,” he said.
He pointed out that Sjögren’s can lead to a lot of disease complications, specifically lymphoma. “Lymphomas are the most important cause of morbidity and mortality in Sjögren’s syndrome patients,” said Dr. Vivino. “This is confusing, but the best predictors of lymphomagenesis include persistent parotid enlargement, persistent lymphadenopathy, palpable purpura, mixed monoclonal cryoglubulinemia, and low complement of C4.”
As for symptoms, there has been recent progress in treating dry eyes. This symptom has greatest quality-of-life effect. Research has shown that inflammation is tied to dry-eye disease. New evidence points to secretagogues, such as pilocarpine and cevimeline, that can alleviate symptoms. Fatty acids seem to play a role as well.