What are the EULAR Guidelines for the management of Sjogren syndrome?

Updated: Mar 05, 2021
  • Author: Sriya K Ranatunga, MD, MPH; Chief Editor: Herbert S Diamond, MD  more...
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Answer

In 2019, the European League Against Rheumatism (EULAR) published guidelines on the management of Sjögren syndrome with topical and systemic therapies. [102]  EULAR recommends that patients with Sjögren syndrome be managed at, or in close collaboration with, centers of expertise following a multidisciplinary approach. Specific recomendations are listed below.

Dry mouth

Baseline evaluation of salivary gland function, including measurement of whole salivary flows, and exclusion of unrelated conditions, is recommended before starting treatment for oral dryness.        

The preferred first therapeutic approach for oral dryness according to salivary gland function may be as follows:

  • Mild dysfunction - Nonpharmacological stimulation (eg, sugar-free acidic candies, lozenges, xylitol, sugar-free chewing gum)
  • Moderate dysfunction - Pharmacological stimulation (eg, pilocarpine, cevimeline; anetholtrithione, bromhexine, N-acetylcysteine in cases of intolerance or non-response)       
  • Severe dysfunction - Saliva substitution  

The guidelines recommend against using any of the following for oral dryness:

  • Hydroxychloroquine
  • Oral glucocorticoids
  • Immunosuppressive agents 
  • Rituximab                            

Dry eyes

The first-line therapeutic approach to ocular dryness includes the use of artificial tears and ocular gels/ointments. Refractory/severe ocular dryness may be managed in stepwise fashion, as follows:

  1. Topical ocular non-steroidal anti-inflammatory drugs (NSAIDs) or corticosteroids may be prescribed by ophthalmologists as a short-term therapeutic approach (maximum 2–4 weeks); if no response or intolerance, then
  2. Topical cyclosporine; if no response or intolerance, then
  3. Serum eye drops; if no response or intolerance, then 
  4. Rescue therapies - Oral muscarinic agonists or plug insertion

Fatigue and/or pain:

  • Concomitant diseases should be evaluated in patients presenting with fatigue/pain, whose severity should be scored using specific tools (eg, EULAR Sjögren's Syndrome Patient Reported Index [ESSPRI], Profile of Fatigue, Brief Pain Inventory).
  • Consider analgesics or other pain-modifying agents for musculoskeletal pain, considering the balance between potential benefits and side-effects.      

Systemic disease:

  • Treatment of systemic disease should be tailored to organ-specific severity using the EULAR Sjögren's syndrome disease activity index (ESSDAI) definitions.
  • Glucocorticoids should be used at the minimum dose and length of time necessary to control active systemic disease (eg, pulses of methylprednisolone followed by doses of 0.5 mg/kg/d or lower as induction therapy in severe presentations, and doses< 0.5 mg/kg/d in moderate/less-severe presentations, with a final target of withdrawing glucocorticoids in patients with inactive disease as soon as possible or at least trying to target a maintenance dose of 5 mg/daily or less with the aid of glucocorticoid-sparing immunosuppressive agents).
  • Immunosuppressive agents (eg, leflunomide, methotrexate, azathioprine, mycophenolate, cyclophosphamide) should be mainly used as glucocorticoid-sparing agents, with no evidence supporting the choice of one agent over another.            
  • B-cell–targeted therapies (eg, rituximab, epratuzumab, belimumab) may be considered in patients with severe, refractory systemic disease.
  • The systemic organ-specific therapeutic approach may follow, as a general rule, the sequential (or combined) use of glucocorticoids, immunosuppressive agents, and biologics.
  • Treatment of B-cell lymphoma should be individualized according to the specific histological subtype and disease stage.

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