eMedicine Specialties > Pediatrics: General Medicine > Rheumatology

Sjogren Syndrome: Treatment & Medication

Author: Eyal Muscal, MD, Assistant Professor, Section of Pediatric Rheumatology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital
Coauthor(s): Marietta Morales de Guzman, MD, Assistant Professor, Department of Pediatrics, Baylor College of Medicine; Consulting Staff, Section of Pediatric Rheumatology, Department of Pediatrics, Texas Children's Hospital, Ben Taub General Hospital; Lawrence K Jung, MD, Chief, Division of Pediatric Rheumatology, Children's National Medical Center; Catherine M Flaitz, DDS, MS, Professor of Oral and Maxillofacial Pathology and Pediatric Dentistry, Department of Diagnostic Sciences, University of Texas Health Sciences Center at Houston, Dental Branch
Contributor Information and Disclosures

Updated: Sep 18, 2009

Treatment

Medical Care

Medical care for children with primary Sjögren syndrome is primarily based on strategies used for adults. No controlled studies in children with this disorder have been reported.

  • Xerostomia - Stimulation of salivary flow with sialagogues, such as pilocarpine (shown to be effective in increasing salivary flow in placebo-controlled randomized adult trials) or cevimeline; mechanical stimulation with sugarless chewing gum or lozenges; topical tissue hydration or lubrication from drinking water or the use of artificial saliva (These measures are supportive yet may improve quality of life in adult populations.)21
  • Oral hygiene
    • Caries control - Good oral hygiene; diet control with attention to decreasing refined sugars, simple carbohydrates, and high-acidity foods and beverages; topical fluorides and remineralizing toothpaste and solutions to enhance remineralization of the teeth (Calcium is leeched from teeth because of reduction in saliva and interaction between simple sugars and acidogenic bacteria.)18
    • Oropharyngeal candidiasis - Topical and systemic antifungal agents; daily cleaning of removable oral prostheses or appliances in older patients
    • Gingivitis and periodontitis - Good plaque control; use of antimicrobial agents such as chlorhexidine gluconate 0.12% oral rinse
  • Keratoconjunctivitis - Artificial tears and conservation of natural tear flow
  • Extraglandular or systemic manifestations - Often require immunosuppressive or disease-modifying antirheumatic drugs (DMARDs) in adults. No standardized immunosuppressive regimen has been established for childhood-onset patients. In one multicenter review, 55% of children received corticosteroids, 17.5% received nonsteroidal anti-inflammatory drugs (NSAIDS), and 12.5% received hydroxychloroquine.8 Hydroxychloroquine was predominantly used for patients in another recent single-institution review.9

Surgical Care

  • In cases of parotid enlargement in adult patients, fine-needle aspiration (FNA) or open biopsy may be required if cystic or neoplastic disease is suspected.

Consultations

  • Ophthalmologist - To diagnose keratitis and uveitis using slit-lamp examination and for supportive care for sicca syndrome and inflammatory changes
  • Dentist - Maintain salivary flow, prevent caries and periodontal disease
  • Rheumatologist - For diagnosis and long-term care of adult or pediatric disease; for evaluation of extraglandular, systemic, or overlapping autoimmune disease symptoms; and to guide use of immunosuppressive medications in extraglandular manifestations
  • Surgeon - For salivary gland biopsy

Diet

A nutritious well-balanced diet with the appropriate servings from the basic food groups is recommended.

  • Patients should drink plenty of fluids with meals to aid in the chewing, tasting, and swallowing. If tolerated, encourage the intake of dairy products, especially low-fat milk, yogurt, and cheese. Milk provides increased oral lubrication, while cheese has a beneficial anticaries effect.
  • Recommend avoiding dry crunchy foods because they are too difficult to swallow and may irritate the mucosa.
  • Patients should avoid spicy or acidic foods and beverages.
  • Patients should avoid simple carbohydrates such as sucrose and highly processed refined foods such as pastries and cookies to decrease the risk of dental caries. If sweetener substitutes are used, monitor the intake because some products may cause abdominal distress.
  • Recommend that patients eat foods at moderate temperatures. Foods can be liquefied or pureed if swallowing is a problem. If increase in calories is needed because of an eating disorder, consider liquid nutritional supplements.
  • Alcoholic beverages and caffeinated drinks, such as coffee, tea, or cola, increase oral dryness. Patients should avoid mouth rinses that contain alcohol because they desiccate the mucosa.

Activity

  • Discourage smoking.
  • Instruct patients to avoid windy and low-humidity environments.
  • The family dwelling should be well humidified.
  • Support normal school attendance and functioning in patients with juvenile Sjögren syndrome.

Medication

Primary Sjögren syndrome usually follows a benign course, and conservative management is indicated. Therapeutic approaches may include increasing lubrication with artificial tears, stimulation of salivary flow with sugar-free gum or lozenges, and 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. Treatment of secondary Sjögren syndrome is determined by the severity of the overlapping autoimmune disorder (might include additional agents such as cyclophosphamide and mycophenolate).

Cholinergic agonists

These agents stimulate salivary secretion.


Pilocarpine (Salagen)

Muscarinic M3 receptor agonist.

Adult

5 mg PO qid

Pediatric

Not established; titrate up from doses of 2.5 mg PO bid

May antagonize effects of anticholinergics (eg, atropine, ipratropium bromide); coadministration with beta-adrenergic antagonists may result in cardiac conduction disturbances

Documented hypersensitivity; uncontrolled asthma; acute iritis; narrow-angle glaucoma

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

May cause sweating, urinary frequency, dizziness, and vasodilatation (ie, flushing); use with caution and close medical supervision in patients with significant cardiovascular disease, Parkinson disease, asthma, or COPD


Cevimeline (Evoxac)

Muscarinic M3 agonist. Has 40-fold less binding affinity to M2 receptors and, thus, has a theoretical benefit of less stimulation to cardiac tissues. Longer duration of action than pilocarpine.

Adult

30 mg PO tid

Pediatric

Not established

May have additive effects when used with other cholinergic agents; concurrent use with beta-blockers may cause potential for cardiac conduction disturbances; CYP2D6 inhibitors (eg, fluoxetine, amiodarone, quinidine, ritonavir, paroxetine) or CYP3A3/4 inhibitors (eg, itraconazole, diltiazem, ketoconazole, verapamil) may increase toxicity; anticholinergic agents (eg, phenothiazines, TCAs, atropine) may decrease effects of cevimeline

Documented hypersensitivity; uncontrolled asthma; acute iritis; narrow-angle glaucoma

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Adverse effects include sweating, nausea, and rhinitis; use with caution with close medical supervision in patients with significant cardiovascular disease, asthma, or COPD

Immunosuppressive agents

These agents are used to treat extraglandular disease (ie, interstitial pneumonitis, glomerulonephritis, polyarthritis, vasculitis, pseudolymphoma, neurologic manifestations).


Prednisone (Deltasone, Meticorten, Orasone, Sterapred)

Corticosteroid with salt-retention properties used for its potent anti-inflammatory effects.

Adult

Up to 60-80 mg PO qd

Pediatric

1-2 mg/kg/d PO

Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Documented hypersensitivity; systemic infections

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Long-term corticosteroid use leads to adrenal insufficiency, which may persist for months after its discontinuation; implement steroid replacement in times of stress and, during that period, avoid exposure to chickenpox and measles; long-term use may impair growth in children; electrolyte and fluid disturbances, myopathy, osteoporosis, vertebral fractures, aseptic necrosis of femoral and humeral heads, peptic ulcer, pancreatitis, esophagitis, facial erythema, skin fragility, impaired wound healing, headache, vertigo, depression, overexcitation, menstrual irregularities, cushingoid features, decreased carbohydrate tolerance, cataracts, or glaucoma may occur

Nonsteroidal anti-inflammatory drugs (NSAIDs)

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.


Naproxen (Aleve, Naprosyn, Naprelan)

For relief of mild to moderate pain. Inhibits inflammatory reactions and pain by decreasing activity of cyclooxygenase, which is responsible for prostaglandin synthesis.

Adult

250-500 mg PO bid (Naprosyn); may increase to 1.5 g/d for limited periods; available in SR formulation (Naprelan)

Pediatric

<2 years: Not established
>2 years: 15-20 mg/kg/d PO divided bid; not to exceed adult dose

Coadministration with aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity of NSAIDs; may decrease effect of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; may increase PT when taking anticoagulants (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; phenytoin levels may be increased when administered concurrently

Documented hypersensitivity; peptic ulcer disease; recent GI bleeding or perforation; renal insufficiency

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Category D in third trimester of pregnancy; acute renal insufficiency, interstitial nephritis, hyperkalemia, hyponatremia, and renal papillary necrosis may occur; patients with preexisting renal disease or compromised renal perfusion risk acute renal failure; leukopenia is rare, is transient, and usually returns to normal during therapy; persistent leukopenia, granulocytopenia, or thrombocytopenia warrants further evaluation and may require discontinuation of drug

Disease-modifying antirheumatic drugs (DMARDs)

These agents are used for polyarthritis not controlled with nonsteroidal anti-inflammatory drugs (NSAIDs). Methotrexate (MTX) has been shown to be effective in managing polyarthritis. Other disease-modifying antirheumatic drugs (DMARDs), such as hydroxychloroquine, sulfasalazine, and D-penicillamine, may be synergistic when coadministered with methotrexate.


Methotrexate (Folex PFS, Rheumatrex)

Unknown mechanism of action in treatment of inflammatory reactions (although may involve adenosine receptors); may affect immune function. Ameliorates symptoms of inflammation (eg, pain, swelling, stiffness).
Adjust dose gradually to attain satisfactory response.

Adult

7.5 mg/wk or 2.5 mg PO/IM bid for 3 doses qwk as per use in RA

Pediatric

10-15 mg/m2/wk PO/SC as a single dose; alternatively, 3 divided doses administered 12 h apart or 2 divided doses 24 h apart

PO aminoglycosides may decrease absorption and blood levels of concurrent PO methotrexate (MTX); charcoal lowers MTX levels; coadministration with etretinate may increase hepatotoxicity of MTX; folic acid or its derivatives contained in some vitamins may decrease response to MTX; coadministration with NSAIDs may be fatal; indomethacin and phenylbutazone can increase MTX plasma levels; may decrease phenytoin serum levels; probenecid, salicylates, procarbazine, and sulfonamides, including TMP-SMZ, may increase effects and toxicity of MTX; may increase plasma levels of thiopurines

Documented hypersensitivity; alcoholism; hepatic insufficiency; documented immunodeficiency syndromes; preexisting blood dyscrasias (eg, bone marrow hypoplasia, leukopenia, thrombocytopenia, significant anemia); renal insufficiency

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Monitor CBC counts monthly and liver and renal function q1-3mo during therapy (monitor more frequently during initial dosing, dose adjustments, or when risk of elevated MTX levels, eg, dehydration); MTX has toxic effects on hematologic, renal, GI, pulmonary, and neurologic systems; discontinue upon significant drop in blood counts; aspirin, NSAIDs, or low-dose steroids may be administered concomitantly with MTX (possibility of increased toxicity with NSAIDs, including salicylates, has not been tested); may consider folic acid supplementation to avoid deficiency

Antimalarial Agent


Hydroxychloroquine

Mechanism of action is unclear; in treatment of inflammatory arthritis, mechanism of action is unknown. May inhibit chemotaxis of eosinophils, locomotion of neutrophils, and impairs complement-dependent antigen-antibody reactions.

Adult

200 mg PO bid

Pediatric

Not established

Serum levels increase with cimetidine; magnesium trisilicate may decrease absorption

Documented hypersensitivity to 4-aminoquinoline derivatives; psoriasis; retinal and visual field changes attributable to 4-aminoquinolines

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in hepatic disease, G-6-PD deficiency, psoriasis, and porphyria; not recommended for long-term use in children; perform periodic ophthalmologic examinations; test for muscle weakness; retinopathy, tinnitus, nerve deafness, skin eruption, headache, anorexia, nausea, vomiting, and diarrhea may occur

More on Sjogren Syndrome

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Differential Diagnoses & Workup: Sjogren Syndrome
Treatment & Medication: Sjogren Syndrome
Follow-up: Sjogren Syndrome
Multimedia: Sjogren Syndrome
References
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Further Reading

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Keywords

Sjogren syndrome, Sjögren's syndrome, Sjögren syndrome, Sicca syndrome, keratoconjunctivitis, xerostomia, polyarthritis, parotitis, salivary gland enlargement, recurrent parotitis, autoimmune exocrinopathy, systemic lupus erythematosus, SLE, rheumatoid arthritis, scleroderma, biliary cirrhosis, lymphoproliferative disease, non-Hodgkin lymphoma, Waldenström macroglobulinemia, B-cell lymphoma, keratoconjunctivitis, Raynaud phenomenon, Epstein-Barr virus, EBV, HIV, cytomegalovirus, treatment, diagnosis

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Contributor Information and Disclosures

Author

Eyal Muscal, MD, Assistant Professor, Section of Pediatric Rheumatology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital
Eyal Muscal, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Rheumatology, and Clinical Immunology Society
Disclosure: Nothing to disclose.

Coauthor(s)

Marietta Morales de Guzman, MD, Assistant Professor, Department of Pediatrics, Baylor College of Medicine; Consulting Staff, Section of Pediatric Rheumatology, Department of Pediatrics, Texas Children's Hospital, Ben Taub General Hospital
Marietta Morales de Guzman, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Rheumatology, and Texas Pediatric Society
Disclosure: Nothing to disclose.

Lawrence K Jung, MD, Chief, Division of Pediatric Rheumatology, Children's National Medical Center
Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Catherine M Flaitz, DDS, MS, Professor of Oral and Maxillofacial Pathology and Pediatric Dentistry, Department of Diagnostic Sciences, University of Texas Health Sciences Center at Houston, Dental Branch
Catherine M Flaitz, DDS, MS is a member of the following medical societies: American Academy of Oral and Maxillofacial Pathology, American Academy of Oral Medicine, American Academy of Pediatric Dentistry, American Dental Association, International Association for Dental Research, and International Association of Oral Pathologists
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

David D Sherry, MD, Director, Clinical Rheumatology, Attending Physician, Pain Management, The Children's Hospital of Philadelphia; Professor of Pediatrics, University of Pennsylvania
David D Sherry, MD is a member of the following medical societies: American College of Rheumatology and American Pain Society
Disclosure: Nothing to disclose.

CME Editor

Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine
Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine
Disclosure: Baxter Honoraria Consulting

 
 
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