Sjogren Syndrome Guidelines

Updated: Aug 18, 2016
  • Author: Anne V Miller, MD; Chief Editor: Herbert S Diamond, MD  more...
  • Print
Guidelines

Guidelines Summary

In 2016, the Sjögren’s Syndrome Foundation (SFF) published clinical practice guidelines for Sjögren disease (SD) patients in the United States. [95] The guidelines note that treatment goals remain as follows:

  1. Symptom palliation
  2. Prevention of complications
  3. For rheumatologists, proper selection of patients for immunosuppressive therapy

Dry mouth

Recommendations (and their strength) for SD patients with dry mouth include the following:

  • Topical fluoride should be used in for caries prophylaxis (strong)
  • Saliva can be increased through gustatory, masticatory stimulation, sugar-free lozenges and/or chewing gum, xylitol, mannitol, and the prescription medications pilocarpine and cevimeline (weak)
  • Chlorhexidine administered by varnish, gel, or rinse may be considered in SD patients with  a high root caries rate (weak)
  • Nonfluoride remineralizing agents may be considered as an adjunct therapy in SD patients with a high root caries rate (moderate)

 

Dry eye

For dry eye disease, recommendations vary according to whether meibomian gland disease is present and by the severity of eye involvement, which is determined mainly by the presence or absence of ocular surface staining and the staining pattern.

Aqueous deficiency without meibomian gland disease

For severity 1 disease, recommendations are as follows:

  • Education and modification of the environment or diet
  • Elimination of offending systemic medication
  • Artificial tears, gels, ointments

For severity 2 disease, recommendations are as follows:

  • Omega 3 essential fatty acid supplement
  • Anti-inflammatory therapy with cyclosporine
  • Anti-inflammatory therapy with pulse steroids
  • Punctal plugs
  • Secretagogues
  • Moisture chamber spectacles

For severity 3 disease, recommendations are as follows:

  • Topical autologous serum
  • Contact lenses
  • Permanent punctal occlusion

For severity 4 disease, recommendations are as follows:

  • Systemic anti-inflammatory medication
  • Eyelid surgery

Aqueous deficiency with meibomian gland disease

For severity 1 disease, recommendations are as follows:

  • Education and modification of the environment or diet
  • Elimination of offending systemic medication
  • Artificial tears with lipid component
  • Eyelid therapy: warm compress, massage

For severity 2 disease, recommendations are as follows:

  • Omega 3 essential fatty acid supplement
  • Anti-inflammatory therapy with cyclosporine
  • Anti-inflammatory therapy with topical steroids
  • Topical azithromycin
  • Liposomal spray
  • Possible oral doxycycline
  • Expression of Meibomian glands
  • Punctal plugs
  • Secretagogues
  • Moisture chamber spectacles

For severity 3 disease, recommendations are as follows:

  • Topical autologous serum
  • Contact lenses
  • Permanent punctal occlusion
  • LipiFlow pulsed thermal compression
  • Probing of meibomian gland

For severity 4 disease, recommendations are as follows

  • Systemic anti-inflammatory medication
  • Eyelid surgery

Inflammatory Musculoskeletal pain

The SFF guidelines advise that physicians consider the individual patient’s circumstances when weighing

risks and benefits of each therapy. Insufficient evidence exists on the effectiveness of disease-modifying antirheumatic drugs (DMARDs) in the treatment of inflammatory musculoskeletal pain in primary SD, so recommendations were formulated on the basis of expert opinion as guided by the consensus group process.

The following recommendations are listed in order of preference for use in the treatment of inflammatory musculoskeletal pain in primary SD; if a therapy is insufficiently effective, the physician is advised to try the next recommendation in sequence, and so on:

  1. Hydroxychloroquine (HCQ); first-line treatment
  2. Methotrexate
  3. HCQ plus methotrexate
  4. Short-term (1 month or less) corticosteroids (15 mg or less a day); long-term corticosteroids (≤15 mg/d for more than 1 month) may be useful, but efforts should be made to find a steroid-sparing agent as soon as possible
  5. Leflunomide
  6. Sulfasalazine
  7. Azathioprine (may be a better choice than leflunomide or sulfasalazine in patients with major organ involvement)
  8. Cyclosporine

 Fatigue

The only strongly recommended treatment of fatigue in SD was exercise.  Hydroxychloroquine may be considered in selected situations

The guidelines include strong recommendations against the use of the following for fatigue:

  • Dehydroepiandrosterone (DHEA)
  • Etanercept
  • Infliximab

Insufficient evidence to issue a recommendation was found for the following therapeutic options for fatigue:

  • Interleukin-1 inhibition (anakinra)
  • Azathioprine
  • Mycophenolate
  • Zidovudine
  • Doxycycline
  • Lamivudine
  • Leflunomide
  • Abatacept
  • Belimumab
  • Epratuzumab

Biological Therapies

The guidelines advise against use of tumor necrosis factor–alpha (TNF-α) inhibitors to treat sicca symptoms in patients with primary SD. If TNF-α inhibition therapy is used for rheumatoid arthritis or other related overlap conditions in patients with SD, health care providers should consider and monitor for the following:

  • Lymphoma and other malignancies (risk for non-Hodgkin is elevated)
  • Serious infections, including tuberculosis
  • Invasive fungal infections
  • Hepatitis B reactivation
  • Hepatotoxicity
  • Heart failure
  • Cytopenias
  • Hypersensitivity
  • Serious infusion reactions
  • Demyelinating disease

Rituximab

Rituximab may be considered as a therapeutic option for the following indications in patients with primary SD:

  • Keratoconjunctivitis sicca (KCS), in patients for whom conventional therapies (eg, topical moisturizers, secretagogues, anti-inflammatory drugs, immunomodulators, punctual occlusion) have proven insufficient
  • Xerostomia, in patients with primary SD with some evidence of residual salivary production and clinician-determined significant evidence of oral damage, in whom conventional therapies, including topical moisturizers and secretagogues, have proved insufficient

Rituximab may be considered as a therapeutic option for adults with primary SD and any or all of the following systemic manifestations:

  • Cryoglobulinemia associated with vasculitis
  • Vasculitis
  • Severe parotid swelling
  •  Inflammatory arthritis
  • Pulmonary disease
  • Peripheral neuropathy, especially mononeuritis

Patients and health care providers should be aware that, although uncommon, significant harms may be associated with the use of rituximab and should exercise caution and observe for the following when using rituximab in patients with SD:

  • Infusion reactions
  • Tumor lysis syndrome in patients with non-Hodgkin lymphoma
  • Progressive multifocal leukoencephalopathy
  • Hepatitis B reactivation with possible fulminant hepatitis
  • Severe mucocutaneous reactions
  • InfectionsBowel obstruction and perforation
  • Cardiac arrhythmias and angina
  • Cytopenias
  • Serious bacterial, viral, or fungal infections

Other recommendations regarding rituximab included the following:

  • Particularly careful consideration of risk versus benefit is necessary in pregnant and nursing patients
  • Live vaccines should be avoided in patients taking rituximab