musculoskeletal and connective tissue disorders
Sjögren Syndrome
Treatment of Sjögren Syndrome
Symptomatic treatment for sicca symptoms Avoidance of aggravating factors Corticosteroids orrituximabfor severe disease Hydroxychloroquineand/ormethotrexatefor musculoskeletal manifestations Sjögren syndrome should be initially managed by topical therapy of dry eyes and dry mouth. Other systemic manifestations of the disease should be treated depending on the severity and the involved organ. Recognition of therapies for other conditions that can exacerbate dryness complaints is crucial. Oralhydroxychloroquine5 mg/kg of body weight once a day is often given to halt the progression of the disease and for the treatment of arthralgias, but supportive trial data are limited. Oralmethotrexate15 to 20 mg once a week can be given for musculoskeletal manifestations. Dry eyes should be treated with lubricating eye preparations (initially drops such as hypromellose ormethylcelluloseand an over-the-counter ointment at bedtime). Other treatments include drainage (punctal) duct closure and topicalcyclosporine. Skin and vaginal dryness can be treated with lubricants. Mouth dryness may be avoided by sipping fluids throughout the day, chewing sugarless gum, and using a saliva substitute containing carboxymethylcellulose as a mouthwash. Drugs that decrease salivary secretion (eg, antihistamines, antidepressants, other anticholinergics) should be avoided. Fastidious oral hygiene and regular dental visits are essential. Stones must be promptly removed, preserving viable salivary tissue. The pain of suddenly enlarged salivary glands is generally best treated with warm compresses and analgesics. Oralpilocarpine5 mg 3 times a day or oralcevimelinehydrochloride 30 mg 3 times a day can stimulate salivary production but should be avoided in patients with bronchospasm and closed-angle glaucoma. Aggressive systemic treatment is occasionally indicated; it is usually reserved for patients with associated diseases (eg, severe vasculitis, optic neuritis, or visceral involvement). Corticosteroids (eg, oralprednisone1 mg/kg once a day), orrituximabmay be needed in severe disease with cryoglobulinemic vasculitis, peripheral neuropathy/multiple mononeuropathy, severe parotid swelling, severe lung involvement, or inflammatory arthritis that does not respond to non-biologic therapy. (See alsotreatment guidelinesfor rheumatologic manifestations of Sjögren syndrome.) Fatigue has no clearly effective treatment. 