Medication Summary
Primary Sjögren syndrome usually follows a benign course in adult patients, and conservative management is indicated. Therapeutic approaches may include increasing lubrication with artificial tears, stimulating salivary flow with sugar-free gum or lozenges, and using vaginal lubricants. Saliva substitutes (eg, carboxymethylcellulose) are not usually effective. Cholinergic agonists have been shown to help increase salivary secretion and are approved by the US Food and Drug Administration (FDA) for this use. The treatment of secondary Sjögren syndrome is determined by the severity of the overlapping autoimmune disorder and may include the use of additional agents, such as methotrexate, azathioprine, cyclophosphamide, rituximab, mycophenolate, mofetil, and other biologic agents.
Cholinergic agonists
Class Summary
These agents stimulate salivary secretion.
Pilocarpine (Salagen)
Pilocarpine is a muscarinic M3 receptor agonist.
Cevimeline (Evoxac)
Cevimeline is a muscarinic M3 agonist. It has 40-fold less binding affinity to M2 receptors and therefore has the theoretical benefit of causing less stimulation of cardiac tissues. This agent has a longer duration of action than pilocarpine.
Immunosuppressive agents
Class Summary
These agents are used to treat extraglandular disease (ie, interstitial pneumonitis, glomerulonephritis, polyarthritis, vasculitis, pseudolymphoma, neurologic manifestations).
Prednisone
Prednisone is a corticosteroid with salt-retention properties used for its potent anti-inflammatory effects.
Nonsteroidal Anti-Inflammatory Drugs
Class Summary
The use of NSAIDs in Sjögren syndrome is similar to agents used for juvenile arthritis. These agents may be used to treat polyarthritis associated with Sjögren syndrome.
Ibuprofen (Motrin, Advil, Caldolor)
Ibuprofen is the drug of choice for patients with mild to moderate pain. It inhibits inflammatory reactions and pain by decreasing prostaglandin synthesis. It also has anti-inflammatory and antipyretic properties. Ibuprofen is available in 200-, 400-, 600-, and 800-mg doses.
Naproxen (Aleve, Naprosyn, Naprelan)
Naproxen is used for the relief of mild to moderate pain. It inhibits inflammatory reactions and pain by decreasing the activity of cyclooxygenase, which is responsible for prostaglandin synthesis.
Disease-Modifying Antirheumatic Drugs
Class Summary
These agents are used for polyarthritis not controlled with nonsteroidal anti-inflammatory drugs (NSAIDs). Methotrexate has been shown to be effective in managing polyarthritis. Other DMARDs, such as hydroxychloroquine, may be synergistic when coadministered with methotrexate.
Methotrexate (Trexall, Rheumatrex)
Methotrexate has an unknown mechanism of action in the treatment of inflammatory reactions (although it may involve adenosine receptors). The drug, which may affect immune function, ameliorates symptoms of inflammation (eg, pain, swelling, stiffness). Adjust the dose gradually to attain a satisfactory response.
Antimalarial Agents
Class Summary
These agents may inhibit chemotaxis of eosinophils and the locomotion of neutrophils and may impair complement-dependent antigen-antibody reactions.
Hydroxychloroquine sulfate (Plaquenil)
The mechanism of action for this drug is unclear; in the treatment of inflammatory arthritis, the mechanism of action is unknown. Hydroxychloroquine sulfate may inhibit chemotaxis of eosinophils and the locomotion of neutrophils and impairs complement-dependent antigen-antibody reactions.
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Lower facial appearance of a 14-year-old adolescent girl with Sjogren syndrome. She exhibits both parotid and submandibular gland enlargement and chapped lips.
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Intraoral view of a 14-year-old adolescent girl with Sjogren syndrome. Hyposalivation results in erythema of the mucosa, gingivitis, decalcification or white spot lesions of the teeth at the cervical margin, and dental caries with extensive restorations of the posterior teeth.
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Erythema of the labial mucosa with enlargement of the minor salivary glands and superficial mucoceles.
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The dorsal surface of the tongue demonstrates generalized atrophy of the filiform papillae, mild fissuring, and median rhomboid glossitis.
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A 14-year-old adolescent girl with Sjogren syndrome with painful unilateral swelling of the knee and hyperpigmentation of the overlying skin.
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The dorsal tongue demonstrates hyperplastic candidiasis with focal erosions and a brown hairy tongue. Ulcerated fissures are observed on the corners of the mouth that represent angular cheilitis.
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Biopsy of the minor salivary glands of the lower lip may be useful in the diagnosis of Sjögren syndrome. A 1.5- to 2-cm incision of normal-appearing mucosa allows for the harvesting of 5 or more salivary gland lobules.
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Low-power photomicrograph of a minor salivary gland lobule showing multiple lymphocytic foci that are replacing the acinar structures (hematoxylin-eosin, 40 X).
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Intermediate-power photomicrograph demonstrating a chronic inflammatory aggregate of more than 50 lymphocytes and plasma cells with a periductal pattern. The inflammatory focus is adjacent to normal appearing acini (hematoxylin-eosin, 200 X).
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High-power photomicrograph of the chronic inflammatory aggregate consists of lymphocytes and plasma cells around a ductal structure (hematoxylin-eosin, 400 X).
