ATLANTA—At the ACR/ARP 2019 Annual Meeting, several widely renowned experts across an array of specialty subjects provided a comprehensive and compelling review of advances in the understanding, diagnosis and treatment of a number of rheumatologic conditions.
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Frederick Vivino, MD, FACR, chief of rheumatology at Penn Presbyterian Medical Center and professor of clinical medicine at the University of Pennsylvania, Philadelphia, discussed important topics related to Sjögren’s syndrome.
Dr. Vivino noted that, in 2016, the ACR and the European League Against Rheumatism (EULAR) developed a set of classification criteria for Sjögren’s syndrome.1 Although these classification criteria are not intended as a direct means of diagnosing the condition, but are meant to serve as criteria for enrollment in clinical trials, they do serve to inform the clinician of what to look for and evaluate in patients suspected of having primary Sjögren’s syndrome.
The criteria are based on the weighted sum of five items: anti-SSA/Ro antibody positivity; focal lymphocytic sialadenitis with a focus score of ≥1 foci/4 mm2; an abnormal ocular staining score of ≥5 (or van Bijsterveld score of ≥4); a Schirmer’s test result of ≤5 mm/5 min; and an unstimulated salivary flow rate of ≤0.1 mL/min.
Dr. Vivino made several important points regarding the use of these criteria, including the importance of having an experienced and meticulous pathologist perform the focus score calculation when evaluating for focal lymphocytic sialadenitis, and that patients with isolated anti-SSB/La antibody positivity should undergo lip biopsy, because these patients are less likely to have Sjögren’s syndrome.
Although clinical, laboratory & imaging signs may point to Behçet’s, the diagnosis is ultimately clinical, without one confirmatory test.
The most potentially mortal complication in Sjögren’s syndrome is the increased risk of lymphoma, and Dr. Vivino noted that approximately 5–7% of patients with Sjögren’s syndrome will develop non-Hodgkin’s B cell lymphoma within 10 years of diagnosis. Thus, rheumatologists must screen for consistent swollen major salivary glands and have a relatively low threshold for performing biopsy in these patients.
Other manifestations of Sjögren’s syndrome, such as dry eyes and dry mouth, frequently rely on symptomatic rather than disease-modifying therapy. It is imperative that patients with dry mouth use sodium fluoride-containing toothpaste to prevent dental caries. With respect to sialogogues, such as pilocarpine and cevimeline, a good general approach is to start at a low dose and slowly titrate upward to avoid side effects, and to instruct patients to take the dose after meals.
For dry eyes, a key distinction is determining if the issue stems from increased evaporation of tears (i.e., Meibomian gland dysfunction) or from decreased production (i.e., tear deficiency), because the treatment varies on the basis of the exact mechanism.
With regard to immunomodulatory or immunosuppressive medications, the evidence supporting efficacy of these treatments has been equivocal. Hydroxychloroquine is commonly used in the treatment of patients with Sjögren’s syndrome; however, a set of treatment guidelines from 2016 noted relatively weak evidence demonstrates this medication is effective in ameliorating fatigue in patients with Sjögren’s syndrome.2
With respect to the treatment of musculoskeletal pain, there is weak evidence for efficacy of leflunomide, sulfasalazine and azathioprine—all of which may be considered after hydroxychloroquine and methotrexate—and rituximab is typically reserved for vasculitis, recurrent or severe parotid gland swelling, recalcitrant arthritis, pulmonary disease and neuropathy.