How are the extraglandular manifestations of Sjogrens Syndrome treated?
Fatigue is a common symptom that may be difficult to alleviate. It is important to rule out hypothyroidism, obstructive sleep apnea, and fibromyalgia, as these conditions have therapies that may result in symptom relief. If coexisting fibromyalgia, it is best to avoid tricyclic antidepressants that may aggravate dryness. Arthritis generally responds to nonsteroidal antiinflammatory drugs, antimalarials, and low doses of prednisone (≤5 mg). Severe extraglandular disease may require higher doses of systemic corticosteroids, azathioprine, mycophenylate mofetil, methotrexate, or cyclophosphamide. Based on case series, rituximab may be beneficial for severe extraglandular manifestations (e.g., vasculitis), but clinical trial data does not support its use for dryness and fatigue. Tumor necrosis factor inhibitors are not helpful. Open-label studies of belimumab and abatacept show promise and good tolerability. Lymphoma should be treated in consultation with an oncologist and based on the type and stage.
