Sjogren's syndrome management: EULAR Guidelines

New consensus-based recommendations from the European League Against Rheumatism (EULAR) address the management of Sjogren's syndrome with topical and systemic therapies.

Sjogren's syndrome presents with a broad spectrum of clinical manifestations and autoantibodies, including antinuclear antibodies (the most frequently detected), anti-Ro/SS-A (the most specific), and cryoglobulins and hypocomplementemia (the main prognostic markers).

For decades, the management of Sjogren's syndrome has been based on symptomatic treatment of sicca symptomatology and broad-spectrum immunosuppression for systemic disease, yet there is little information on the relative efficacy and safety of the available therapeutic options.

Dr Manuel Ramos-Casals from Hospital Clinic of Barcelona, in Spain, and colleagues on the EULAR-Sjogren Syndrome Task Force reviewed the existing evidence and used a Delphi procedure to develop consensus recommendations for the management of Sjogren's syndrome.

Three general principles underlie a dozen specific recommendations:

• Patients with Sjogren's syndrome should be managed at, or in close collaboration with, centres of expertise following a multidisciplinary approach;


• The first therapeutic approach for dryness should be symptomatic relief using topical therapies; and


• Systemic therapies may be considered for the treatment of active systemic disease.

After evaluation of salivary gland function, treatment for oral dryness should include nonpharmacological stimulation for mild dysfunction, pharmacological stimulation for moderate dysfunction, and saliva substitutes for severe dysfunction, the authors advise.

Ocular dryness should be treated first with artificial tears and ocular gels/ointments, followed by topical immunosuppressive-containing drops and autologous serum eye drops for refractory or severe ocular dryness.

Musculoskeletal pain can be managed with analgesics or other pain-modifying agents while balancing their potential benefits and side effects.

Treatment of systemic disease should be tailored to organ-specific severity using the EULAR Sjogren's syndrome disease activity index (ESSDAI) definitions, according to the report, online October 31 in Annals of the Rheumatic Diseases.

As a general rule, the systemic organ-specific therapeutic approach should consist of the sequential or combined use of glucocorticoids, immunosuppressive agents, and biologics.

"The Task Force is convinced that adhering to these recommendations, including shared decision-making, assessing disease activity regularly with the ESSDAI instrument, and applying the sequence of drugs as proposed, will improve overall outcomes in a clear majority of patients with Sjogren's syndrome," the authors conclude.

"New research information on treatment strategies, predictive markers, and other aspects will soon become available and will probably require an update of the recommendations in coming years," they add. "Until then, we hope that the current recommendations will be broadly applied in clinical practice and/or serve as a template for national societies to develop local recommendations."

Dr Alan Baer from Johns Hopkins University School of Medicine, in Baltimore, MD, who studies Sjogren's, told Reuters Health by email, "Sjogren's is a disease with a high symptom burden for many patients and potentially serious complications for a minority. There should be an emphasis on accuracy of diagnosis, comprehensive evaluation, and evidence-based approaches to treatment."

While he agrees that patients can benefit from management at expert centres, he notes that "such centres are rare in the United States, and specialists with the requisite expertise are few in number. Thus, it may prove impractical to have all Sjogren's patients managed in speciality centres."

Dr Baer added, "The recommendations are hindered by the need to rely on data from limited clinical trials. The results of such trials may not capture impressive anecdotal data. As an example, many of my patients with severe dry eye gain dramatic relief from autologous serum tears. Similarly, I would expect many ophthalmologists would argue that punctal plug insertion is beneficial and should be tried earlier in the therapeutic algorithm (i.e., before the use of expensive autologous serum tears)."