Biological Therapies

Recommendation 1: TNF-α inhibitors

TNF-α inhibitors should not be used to treat sicca symptoms in patients with primary SD. Strength of recommendation: strong

Recommendation 2: TNF-α inhibitor cautions

If TNF-α inhibition therapy is used for RA or other related overlap conditions in SD patients, health care providers should consider and monitor for the following: Lymphoma and other malignancies; health care providers should be cognizant that patients with primary SD have an increased risk of non-Hodgkin lymphoma compared with the general population Serious infections, including tuberculosis Invasive fungal infections Hepatitis B reactivation Hepatotoxicity Heart failure Cytopenias Hypersensitivity, serious infusion reactions Demyelinating disease Strength of recommendation: strong

Recommendation 3: Rituximab for keratoconjunctivitis sicca (KCS)

Rituximab may be considered as a therapeutic option for KCS in patients with primary SD and for whom conventional therapies, including topical moisturizers, secretagogues, anti-inflammatories, immunomodulators, and punctual occlusion, have proven insufficient. Strength of recommendation: weak

Recommendation 4: Rituximab for xerostomia

Rituximab may be considered as a therapeutic option for xerostomia in patients with primary SD with some evidence of residual salivary production, significant evidence of oral damage as determined by the clinician, and for whom conventional therapies, including topical moisturizers and secretagogues, have proven insufficient. Strength of recommendation: weak

Recommendation 5: Rituximab for systemic symptoms

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 Strength of recommendation: moderate

Recommendation 6: Rituximab cautions

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 SD patients: Infusion reactions Tumor lysis syndrome in patients with non-Hodgkin lymphoma Progressive multifocal leukoencephalopathy Hepatitis B reactivation with possible fulminant hepatitis Severe mucocutaneous reactions Infections Bowel obstruction and perforation Cardiac arrhythmias and angina Cytopenias Serious bacterial, viral, or fungal infections In pregnancy and nursing, the risk vs benefit must be carefully considered Health care providers should avoid giving live vaccines when patients are on rituximab. Strength of recommendation: strong