Sj gren's disease Management Exercise and self-care Provide education regarding self-care measures, including advice about exercise to reduce fatigue in patients with SD. A Management of fatigue SSF Q Does the patient have fatigue related to Sj gren's disease? Yes Consider offering hydroxychloroquine for the management of fatigue in selected patients with SD. C Do not use the following medications for the management of fatigue in patients with SD: etanercept infliximab DHEA. D No Management of keratoconjunctivitis sicca SSF Q Does the patient have keratoconjunctivitis sicca? Yes Consider offering conventional therapies including topical moisturizers, secretagogues, anti-inflammatories, immunomodulators, and punctal occlusion in patients with primary SD and keratoconjunctivitis sicca. C Consider offering rituximab as a therapeutic option in patients with primary SD and keratoconjunctivitis sicca if conventional therapies have proven insufficient. C Do not use TNF inhibitors for the management of sicca symptoms in patients with primary SD. D No Management of xerostomia SSF Q Does the patient have xerostomia? Yes Offer topical fluoride for the management of dry mouth in patients with SD. A Offer the following agents for gustatory, masticatory stimulation, and pharmaceutical agents to increase saliva for the prevention of caries in patients with SD and dry mouth: sugar-free lozenges chewing gum xylitol mannitol pilocarpine cevimeline. B Consider offering rituximab as a therapeutic option for the management of dry mouth in patients with primary SD if there is evidence of residual salivary production, significant evidence of oral damage as determined by the clinician, and if conventional therapies, including topical moisturizers and secretagogues, have proven insufficient. C Do not use TNF inhibitors for the management of sicca symptoms in patients with primary SD. D No Management of xerostomia SSF Q Does the patient have a high root caries rate? Yes Consider offering non-fluoride remineralizing agents as adjunct therapy in patients with SD having dry mouth and a high root caries rate. C Consider offering chlorhexidine administered by varnish, gel, or rinse in patients with SD having dry mouth and a high root caries rate. C No Management of musculoskeletal pain First-line Q Does the patient have inflammatory musculoskeletal pain? Yes Offer hydroxychloroquine monotherapy for the management of inflammatory musculoskeletal pain in patients with primary SD. B Consider offering methotrexate monotherapy for the management of inflammatory musculoskeletal pain in patients with primary SD not responding to hydroxychloroquine. C Consider offering a combination of methotrexate and hydroxychloroquine if either monotherapy is not effective. C No Consider administering short-term corticosteroids for the management of inflammatory musculoskeletal pain in patients with primary SD not responding to combination of methotrexate and hydroxychloroquine. B Management of musculoskeletal pain Second-line Q Does the patient's musculoskeletal pain respond to combination methotrexate and hydroxychloroquine? No Consider administering short-term corticosteroids for the management of inflammatory musculoskeletal pain in patients with primary SD not responding to combination of methotrexate and hydroxychloroquine. B Yes Management of systemic manifestations SSF Q Does the patient have evidence of vasculitis? Q Does the patient have severe parotid swelling? Q Does the patient have inflammatory arthritis? Q Does the patient have SD-related pulmonary disease? Q Does the patient have SD-related peripheral neuropathy? Yes to any Consider offering rituximab as a therapeutic option in adult patients with primary SD and any of the following systemic manifestations: severe parotid swelling vasculitis (with or without cryoglobulins) inflammatory arthritis pulmonary disease peripheral neuropathy, especially mononeuritis. C No to all Monitoring of patients on rituximab Q Will the patient be receiving rituximab? Yes Monitor for the following adverse effects in patients receiving rituximab: 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. B Routine immunizations Avoid giving live vaccines in patients receiving rituximab. D No Continue management and follow-up as clinically appropriate.