Successful Sjögren’s syndrome diagnosis and management requires a multi-disciplinary team and continues to be the most challenging of the rheumatic diseases to diagnose, according to two Sjögren’s specialists who provided clinical pearls of wisdom in Sunday’s clinical session Sjögren’s Syndrome: Beyond Dryness: A Comprehensive Approach to Diagnosis and Management.
Alan N. Baer, MD, Professor of Medicine and Director of the Jerome L. Greene Sjögren’s Syndrome Center at Johns Hopkins University School of Medicine, discussed the challenges of diagnosing Sjögren’s.
“An accurate diagnosis is really a fundamental prerequisite for appropriate management of a patient with existing and emerging targeted therapies, so if we are going to make progress in this field, it is really essential that we diagnose this accurately,” Dr. Baer said.
That accurate diagnosis involves not only the rheumatologist, but also an ophthalmologist, and an oral surgeon or ENT specialist.
“We also have to push our ophthalmology and pathology colleagues to do their part of the assessments according to protocols,” he said.
Dr. Baer reviewed the diagnostic flow from initial suspicion of Sjögren’s to clinical examination for signs of salivary or tear hypofunction and/or salivary or lacrimal gland abnormality using Schirmer’s test, sialometry, and ultrasonography, all of which a rheumatologist can perform in the office. Based on the rheumatologist’s testing, a referral to an eye care professional for Schirmer’s test and slit lamp with ocular surface staining or a labial gland biopsy by an oral surgeon may also be indicated.
“I recognize that the existing diagnostic tests are imperfect, and the results clearly require careful interpretation,” he said.
Arthur A.M. Bookman, MD, FRCPC, Coordinator of the Multidisciplinary Sjogren’s Clinic at University of Toronto, Canada, also emphasized the importance of the multidisciplinary approach to diagnosis to rule out differential diagnoses. The rheumatologist’s role is to coordinate the team.
The management challenge of Sjögren’s is in the limited benefit drugs have in treating the dry eyes and dry mouth caused by the syndrome.
“One major point is that there isn’t always a very good correlation between your objective measures of dryness in your patient and the patient’s actual complaints of dryness,” said Dr. Bookman.
You have to use local measures, he added, such as artificial tears products. He reviewed the different compositions of artificial tear products, as well as several studies on the use of cyclosporine, lifitegrast and steroid drops for dry eyes.
“One of the most useful vehicles that we find in our Sjögren’s clinic is autologous serum drops. The full story I don’t think is in, yet. We’re not sure why they help so much, but they can help people,” he said.
Dr. Bookman also reviewed the drug-related causes of dry mouth and the research on saliva-producing treatments along with promising research on parasympathetic stimulants, including trials of pilocarpine tablets and rituximab, and less convincing results for hydroxychloroquine.
“There was a symptomatic improvement with pilocarpine. The most common side effect was sweating and urinary frequency, so you should be aware of that when you’re prescribing,” said Dr. Bookman.