AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Background

Sjögren syndrome is a slowly progressive inflammatory disorder that involves the exocrine glands. Mikulicz and others recognized the findings of keratoconjunctivitis and xerostomia as an entity in the late 1800s. In 1933, Sjögren recognized the association of this symptom complex with polyarthritis. Subsequent studies showed that Sjögren syndrome may be a primary disorder or may be secondary to other autoimmune disorders, such as systemic lupus erythematosus (SLE), rheumatoid arthritis, scleroderma, and biliary cirrhosis. Extraglandular manifestations may mimic these other autoimmune disorders. The primary disorder is most common in women in the fourth and fifth decades of life. Sjögren syndrome may overlap with other pediatric autoimmune disorders and, less commonly, may present as a primary condition.

Pathophysiology

Lymphocytic infiltration in the glandular lobules is the characteristic abnormality in lacrimal or salivary gland biopsy findings. The disorder appears to be mediated by a complex cascade of immune events and is often characterized as an autoimmune exocrinopathy.

The predominant immune cells are activated memory CD4⁺ T cells that express alpha/beta T-cell receptors, suggesting a central role in the pathogenesis of this disorder. Activated B cells are also found in the lesions, which may be responsible for the production of the autoantibodies to autoantigens Ro (SS-A) and La (SS-B). Anti-Ro (SS-A) is found in 40-45% of adult patients with Sjögren syndrome, and anti-La (SS-B) is found in 50% of these patients. Sixty-three percent of pediatric patients have one of these autoantibodies. Because these autoantibodies are also observed in other autoimmune disorders, including SLE, further diagnostic tools must be used for a more definitive diagnosis.

Family members of these patients have a higher incidence of Sjögren syndrome. The association of Sjögren syndrome with human leukocyte antigen (HLA)–B8, HLA-Dw3, HLA-DR3, and with the DQA1*0501 allele supports the notion of genetic susceptibility.

An active area of research involves genetic polymorphisms of cytokine genes, including IL10 and TNFA. Associations with these polymorphisms might implicate inappropriate regulation of the immune response in Sjögren syndrome.

Altered apoptotic mechanisms have also been implicated in the pathogenesis of Sjögren syndrome–related exocrinopathies. Exocrine gland tissue damage and chronic inflammation leads to fibrotic changes and impaired glandular function.

The pathophysiology of extraglandular manifestations is thought to be due to similar immune mechanisms.

Frequency

United States

Although Sjögren syndrome has been recognized in patients of all ages, it primarily affects women in the fourth and fifth decades of life. It is rare in childhood, although over 130 cases of primary disease have been described in the literature. Five percent of adult patients report onset of symptoms before age 12 years. Clinical manifestations and classification schema differ in pediatric patients; therefore, this disorder may be underreported. Features of Sjögren syndrome are well described in pediatric patients who have other autoimmune disorders. Overall, Sjögren syndrome is the second most common autoimmune disorder in adults, after rheumatoid arthritis. In the United States, between 500,000 and 2 million adults have this disease.

International

Epidemiological studies from different ethnic groups show prevalence rates similar to those in the United States.

Mortality/Morbidity

• Although most patients have a mild and benign course, they often experience painful eye irritation, severe dental caries, and dyspareunia. Pediatric patients often do not have overt sicca syndrome symptomatology but often have recurrent parotitis. Because of the insidious nature of these symptoms, patients often do not seek medical attention until more severe symptoms appear years later. The time from disease onset until diagnosis is often 9 years in adults and perhaps up to 3 years in pediatric patients. Some patients develop systemic features that involve the nervous system, kidney, and lungs.
• Lymphoproliferative disorders increase 40-fold in adult Sjögren syndrome. Although significant lymphoproliferation usually remains confined to the salivary and lacrimal glands, extraglandular lymphoproliferation (eg, lymphadenopathy, hepatosplenomegaly) sometimes resembles lymphoma (eg, pseudolymphoma) but may herald frank malignancies, including non-Hodgkin lymphoma, Waldenström macroglobulinemia, and B-cell lymphoma.Associations with lymphoproliferative diseases are unclear in pediatric populations.

Sex

The female-to-male ratio is approximately 9:1 in adults and 7:1 in one multicenter international pediatric cohort (Anaya, 1995; Cimaz, 2003).

Age

Sjögren syndrome is relatively rare in childhood. Disease onset in primary juvenile Sjögren syndrome appears to occur before age 10 years. The peak incidence in adult populations is around the fourth and fifth decades of life.

CLINICAL

Section 3 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
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History

• Sicca syndrome
• • Keratoconjunctivitis: Symptoms include dry eyes with reduced tear production, gritty or sandy sensation under the lids, red eyes, and photosensitivity. Keratoconjunctivitis is less prominent in primary juvenile Sjögren syndrome. Lacrimal gland enlargement appears to be a feature in both primary and secondary pediatric Sjögren syndrome.
  • Xerostomia: Symptoms include decreased saliva production that leads to difficulties with chewing, swallowing, and even speech; abnormality in taste and smell; dental caries; mucosal burning sensation; sensitivity to spicy and acidic foods and beverages; increased risk for oral candidiasis; hoarseness of voice, and dysphonia (common in adults). Recurrent parotitis appears to be the most common oroglandular manifestation in pediatric populations.
• Musculoskeletal symptoms
• • Arthralgia (often noninflammatory), morning stiffness, nonerosive arthritis
  • Myalgia, muscle weakness
• Cutaneous findings
• • Raynaud phenomenon
  • Nonthrombocytopenic purpura, especially of lower extremities
  • Nasal, vaginal, and cutaneous dryness
• Gastrointestinal symptoms
• • Dysphagia, nausea, epigastric, and abdominal pain
  • Achalasia (in children)
  • Achlorhydria, chronic atrophic gastritis (adult patients)
  • Primary biliary cirrhosis
• Pulmonary findings
• • Dyspnea due to mild interstitial disease
  • Dry cough
• Renal findings - Interstitial nephritis and renal tubular acidosis
• Other symptoms - Fatigue, depression, and insomnia

Physical

• Parotid gland enlargement and recurrent parotitis (pediatric populations)
• Corneal ulceration, vascularization, uveitis
• Vasculitic lesions - Purpura and erythema nodosum
• Lymphadenopathy
• Autoimmune thyroiditis
• Nervous system manifestations
• • Peripheral sensorimotor neuropathy
  • CNS disorders such as movement disorder, transverse myelopathy, encephalopathy, aseptic meningitis, dementia, optic neuropathy, and cranial neuropathies (in both adult and pediatric patients)
• Musculoskeletal manifestations
• • Intermittent synovitis
  • Chronic nonerosive oligoarticular or polyarthritis (Jaccoud arthropathy is observed in adults.)
  • Myalgias
• Oral cavity manifestations
• • Mild erythema and thinning of the mucosa
  • Dental caries
  • Traumatic erosions and ulcers, angular cheilitis, and chapped lips
  • Frothy, ropey, and thickened saliva
  • Erythema, fissuring, coating, and depapillation of the dorsal tongue
  • Halitosis

Causes

Multiple etiological factors are likely involved in the pathogenesis of Sjögren syndrome.

• Sex hormone: As with other autoimmune disorders, Sjögren syndrome is more common in females. This suggests that sex hormones may play a role in shaping the autoimmune response.
• Genetics: The association of certain HLAs suggests that genetic factors likely play a role in the pathogenesis of Sjögren syndrome. Patients with HLA-B8, HLA-Dw3, and HLA-DR3 have a high incidence of autoantibody production and extraglandular manifestations. Most patients also have the DQA1*0501 allele, which may play a role in the pathogenesis of this disease.
• Environmental factors: Epstein-Barr virus (EBV) replicates in the salivary glands during primary infection and remains latent in these organs. EBV-DNA is recovered from salivary glands and saliva of these patients. Its etiopathological role cannot be proven. Human immunodeficiency virus (HIV), human T-cell leukemia-lymphoma virus type 1 (HTLV-1), and cytomegalovirus (CMV) are also under scrutiny as possible inciting agents that lead to lymphoproliferation observed in the end organs.
• Autoantibodies: The presence of anti-Ro (SS-A) and anti-La (SS-B) is associated with earlier onset, longer duration, and more extensive extraglandular manifestations of primary Sjögren syndrome. Antinuclear antibody (ANA) and rheumatoid factor (RF) are prevalent in both pediatric and adult cohorts. Autoantibody production and evidence of a polyclonal gammopathy implicate B-cell dysfunction as a component of Sjögren syndrome pathogenesis. Increased levels of B-cell activating factor (BAFF) or B-lymphocyte stimulator (BLyS), which are essential for B-cell survival, can be found in patients with Sjögren syndrome. Thus, B-cell depletion therapies that include the anti-CD20 monoclonal antibody (rituximab) are being used in open-label studies.
• Inflammatory reactivity: In situ evidence suggests that proinflammatory cytokines, interleukin (IL)–1, IL-6, and tumor necrosis factor (TNF)–alpha are produced in the salivary glands. These cytokines are found in the infiltrating lymphocytes and the epithelial cells. This suggests that the infiltrating lymphocytes (ie, predominantly CD4 T cells) play a role in the perpetuation of an aberrant immune response. Specific IL-10 haplotypes are associated with early onset and vasculitic features of disease. Additional cytokine and chemokine gene polymorphisms are currently being studied.

DIFFERENTIALS

Section 4 of 11  

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• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Other Problems to be Considered

Medications that cause sicca symptoms (anticholinergic)
Recurrent juvenile parotitis
Oropharyngeal candidiasis
Gingivitis/periodontitis
Retrograde bacterial sialadenitis
Halitosis
Superficial mucoceles
Compromised nutrition
Dryness of the nasal and vaginal mucosa and skin
Pregnancy complications due to autoantibodies

WORKUP

Section 5 of 11  

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• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
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Lab Studies

• CBC count with differential: Mild anemia and leukopenia are present.
• Erythrocyte sedimentation rate (ESR): Elevated ESR is observed in 80-90% of patients, often related to hypergammaglobulinemia; however, C-reactive protein (CRP) levels are usually within the reference range.
• Immunoglobulin levels: Hypergammaglobulinemia, up to several grams of immunoglobulin G (IgG), is observed in 70-80% of both pediatric and adult patients.
• Autoantibodies
• • ANA and RF levels - Usually elevated
  • Anti-Ro (SS-A), anti-La (SS-B)
  • Autoantibodies to thyroglobulin, thyroid microsomal, mitochondrial, smooth muscle, parietal, peroxisomal, and salivary duct (often present in adults with Sjögren syndrome)
  • Cryoglobulins and, occasionally, antiphospholipid antibodies
• Rose Bengal staining: The dye stains damaged corneal epithelium and indicates keratoconjunctivitis. This is not often performed in children.
• Schirmer tear test: This test is used to evaluate tear production by lacrimal glands. A strip of filter paper is placed beneath the lower lid, and wetting of the paper is measured at 5 minutes. Less than 10 mm of film is abnormal, and less than 5 mm of wetting suggests decreased tear production and sicca syndrome. This test is performed more often in pediatric patients.

Imaging Studies

• Sialography - A sensitive and specific radiographic technique to find evidence of sialectasis (Sialography sensitivity has not been reported in the pediatric literature. These techniques are not commonly used at most pediatric centers.)
• Technetium Tc 99 pertechnetate scintigraphy - Delayed uptake in Sjögren syndrome; often correlates with pathological changes
• MRI - Visualization of the glandular parenchyma, in particular for the evaluation of cystic or solid masses (In addition, the volumetric estimate of the gland size can be determined.)

Other Tests

• In the questionnaire of adult salivary hypofunction, positive responses to all 4 of the following questions indicate major salivary gland hypofunction (adapted from Fox, 1998).
  • Do you sip liquids to aid in swallowing dry foods?
  • Does your mouth feel dry when eating a meal?
  • Do you have difficulties swallowing any foods?
  • Does the amount of saliva in your mouth seem to be too little?
• Sialometry is the quantification of whole saliva or individual gland secretions at unstimulated (resting) or stimulated flow rates. For salivary hypofunction, the flow rate for unstimulated whole saliva is less than 0.1 mL/min, while the rate for stimulated whole saliva is less than 0.5 mL/min. The collection period is a minimum of 5 minutes and often up to 15 minutes. When secretions from the parotid gland are evaluated, the modified Carlson-Crittenden collector is placed over the Stensen duct. Isolation of the salivary gland orifices in the floor of the mouth and gentle suction are used to collect submandibular and sublingual secretions together. Besides demonstrating salivary hypofunction, these methods can be used to evaluate the effectiveness of secretogogue therapy. These procedures are not commonly used in pediatric patients.
• On sialochemistry, collected secretions can be chilled, frozen, and evaluated for electrolytes, immunoglobulins, and protein constituents. Although not diagnostic for Sjögren syndrome, a profile has been observed, including an increase in secretory levels of immunoglobulin A, lactoferrin, total protein, and sodium and chloride ions. In addition, decreased levels of lysozyme and potassium and phosphate ions are found. Although still considered experimental, these changes in salivary constituents are of unknown predictive value.
• Less than 50% of pediatric patients report ocular symptoms, whereas older patients with Sjögren syndrome more frequently report them. Ocular screening questions include the following:
• • Have you had persistent dry eyes daily for more than 3 months?
  • Do you have recurrent sensation of sand or gravel in your eyes?
  • Do you use tear substitutes more than 3 times daily?

Procedures

• Salivary gland biopsy
• • The pathological findings are very useful in diagnosis and are often obtained while diagnosing juvenile primary Sjögren syndrome.
  • Because of the relative ease and lack of complications, labial minor salivary gland biopsy is preferred over parotid gland biopsy, which can result in facial nerve damage. However, minor salivary gland biopsy requires sufficient expertise to ensure adequacy of tissue collection.
  • In order to ensure that a representative sample has been obtained for histopathologic examination, harvesting 5-10 lobules of minor salivary glands is crucial.

Histologic Findings

The characteristic histopathologic findings of the minor salivary glands include an inflammatory infiltrate adjacent to normal-appearing acinar structures. The inflammatory infiltrate consists of primarily lymphocytes and fewer plasma cells. Most of the infiltrating lymphocytes are activated CD4⁺ memory T lymphocytes. A focal periductal pattern is initially observed, with eventual confluence of the inflammatory infiltrate that replaces the acini. Periductal and perivascular hyaline deposits may be observed.

Unlike the parotid gland lesions, epimyoepithelial islands are rarely observed in the lymphocytic background. The finding of more than one focus of 50 or more inflammatory cells within a 4-mm² area of glandular tissue supports the diagnosis of Sjögren syndrome. The greater the number of foci, the greater the disease correlation. A negative biopsy finding cannot completely exclude a diagnosis of Sjögren syndrome. Biopsies may also be used to rule out sarcoid or amyloid lesions.

When the major glands are enlarged, a benign lymphoepithelial lesion (BLEL) may develop. The characteristic features include a dense lymphocytic infiltrate that is associated with the destruction of salivary gland acini, while the ductal epithelium persists. Hyperplasia of the ductal epithelium and myoepithelial cells forms epimyoepithelial islands within the lymphoid tissues. Formation of germinal centers may be observed. Determination of monoclonality of the lymphocytic infiltrate by immunohistochemical or gene rearrangement studies may be necessary in order to exclude a low-grade B-cell lymphoma in adult patients.

Staging

• Diagnostic and classification schema: Diagnosis is made if the serology or histopathology is positive and if 4 of the following American-European Consensus Group (AECG) criteria for adult patients are met:
• • At least one positive response to the ocular symptoms questions (see Other Tests)
  • At least one positive response to the oral symptoms questions (see Other Tests)
  • Ocular signs (positive Schirmer tear test or Rose-Bengal stain findings)
  • Histopathological features on minor salivary gland biopsy findings
  • Salivary gland involvement revealed by at least one testing modality (salivary scintigraphy, parotid sialography, unstimulated salivary flow)
  • Anti-Ro (SS-A) or anti-La (SS-B)
  • Exclusion criteria - Preexisting lymphoma, HIV, hepatitis C, sarcoidosis, graft-versus-host disease (GVHD)
• Proposed criteria for pediatric patients have not been prospectively validated. Only 76% sensitivity has been noted in compilation of retrospective patients. The clinical judgment of a pediatric rheumatologist is the criterion standard. However, the proposed pediatric criteria appear more sensitive than adult AECG criteria in classifying pediatric Sjögren syndrome. Diagnosis is based on the presence of 4 or more of the following proposed pediatric diagnostic criteria:
• • Clinical symptoms
    • Oral symptoms (dry mouth, parotitis, parotid gland enlargement)
    • Ocular symptoms (recurrent conjunctivitis, keratoconjunctivitis sicca)
    • Other mucosal symptoms (recurrent vaginitis, vulvovaginitis)
    • Systemic symptoms (fever of unknown origin, noninflammatory arthralgias, hypokalemic paralysis)
  • Immunologic abnormalities - Presence of anti-Ro (SS-A), anti-La (SS-B), high titer ANA, or RF
  • Other laboratory finding abnormalities or additional investigations
    • Elevated serum amylase levels
    • Leukopenia, high ESR
    • Polyclonal hyperimmunoglobulinemia
    • Renal tubular acidosis
    • Histological proof of lymphocytic infiltration of salivary gland or other organs
    • Objective documentation of ocular dryness (Rose Bengal stain findings, Schirmer tear test findings)
    • Objective documentation of parotid gland enlargement (sialography findings)
  • Exclusion of all other autoimmune disease

TREATMENT

Section 6 of 11  

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Medical Care

• 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.)
• 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.)
  • 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)

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

Section 7 of 11  

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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).

Drug Category: Cholinergic agonists

These agents stimulate salivary secretion.

Drug Category: Immunosuppressive agents

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

Drug Category: 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.

Drug Category: Disease-modifying antirheumatic drugs (DMARDs)

These agents are used for polyarthritis not controlled with NSAIDs. Methotrexate (MTX) has been shown to be effective in managing polyarthritis. Other DMARDs (eg, hydroxychloroquine, sulfasalazine, D-penicillamine) may be synergistic when coadministered with methotrexate.

FOLLOW-UP

Section 8 of 11  

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• Follow-up
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Further Inpatient Care

• Hospitalization, significant morbidities, and use of immunosuppressive medications are dictated by systemic extraglandular features.
• Overlapping symptoms of SLE include cerebritis, nephritis, and severe Raynaud phenomenon. Severe systemic manifestations may also develop without overt features of other autoimmune disorders.

Further Outpatient Care

• Routine follow-up care by a rheumatologist, ophthalmologist, and dentist

Deterrence/Prevention

• Caries prevention
• • Personal dental plaque measures include twice-daily cleaning of the teeth with a toothbrush, using a fluoride-containing dentifrice, and daily use of dental floss; increase the number of professional cleanings to 3-4 times a year if carious lesions develop.
  • Daily home use of topical fluorides, especially gel or toothpaste that contains 1.1% sodium fluoride or remineralizing gel with 0.05% sodium fluoride, sodium phosphate, and calcium carbonate, is recommended.
  • If the patient has severe xerostomia, use custom fluoride trays or carriers to apply the topical fluorides.
  • Use chlorhexidine gluconate oral rinse concurrently for 2-week periods when high numbers of Streptococcus mutans are found in the saliva (>1 X 10⁶/mL saliva).
  • Limit the intake of sugary food and beverages between meals. Use sweetener alternatives, if tolerated, such as aspartame, saccharin, sorbitol, and xylitol.
• Prevention of oral mucosal lesions
• • Chapped lips - Repeated use of water- or lanolin-based lip moisturizers (Avoid lip products that are medicated with menthol or phenol because they cause further drying.)
  • Traumatic erosions and ulcers - Frequent hydration and the use of artificial saliva or oral moisturizing agents, especially under removable oral prostheses (see Diet)
  • Oropharyngeal candidiasis - Good oral hygiene, frequent oral hydration and lubrication, and nightly removal and cleaning of dental prostheses (Intermittent use of topical or systemic antifungal agents may be necessary to prevent recurrent infection. If topical antifungal agents are used, consultation with a compounding pharmacist is recommended in order to formulate sucrose-free suspensions or lozenges.)

Complications

• Adult patients have a small risk of developing lymphoma and other malignancies.

Prognosis

• Patients with primary Sjögren syndrome usually have a good prognosis unless severe extraglandular manifestations appear. The prognosis of secondary Sjögren syndrome depends on the primary autoimmune disorder.

Patient Education

• Because this is a chronic disease, participation in a local or national support group may be beneficial. The Sjögren's Syndrome Foundation is an example of an organization that helps individuals cope with this disease. Additional resources can be found at The Lupus Foundation of America and the Arthritis Foundation.
• For excellent patient education resources, visit eMedicine's Arthritis Center. Also, see eMedicine's patient education article Sjogren Syndrome.

MISCELLANEOUS

Section 9 of 11  

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• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• Failure to diagnose and, thus, improve impairments of quality of life caused by sicca syndrome
• Failure to recognize that adult patients have a small risk of developing lymphoma and other malignancies
• Failure to review medication risks carefully with the patient and family prior to treatment with any new medication
• Failure to diagnose systemic extraglandular manifestations of Sjögren syndrome or presence of a primary autoimmune disorder (eg, SLE) that requires immunosuppressive agents

Special Concerns

• Many of the symptoms associated with this disorder can seriously impair an individual's quality of life. In addition to sicca syndrome, concerns about facial appearance, depression, chronic fatigue, and joint pain must be addressed. Parotid enlargement and weight gain (if corticosteroids are used to manage the disease) may be problematic in an adolescent.
• Close attention must be paid to emotional and cognitive functioning of the adolescent coping with a chronic disease.

MULTIMEDIA

Section 10 of 11  

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• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  Lower facial appearance of a 14-year-old adolescent girl with Sjögren syndrome. She exhibits both parotid and submandibular gland enlargement and chapped lips.
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Media file 2:  Intraoral view of a 14-year-old adolescent girl with Sjögren syndrome (see Image 1). 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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Media file 3:  Erythema of the labial mucosa with enlargement of the minor salivary glands and superficial mucoceles.
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Media file 4:  The dorsal surface of the tongue demonstrates generalized atrophy of the filiform papillae, mild fissuring, and median rhomboid glossitis.
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Media file 5:  A 14-year-old adolescent girl with Sjögren syndrome (see Image 1) with painful unilateral swelling of the knee and hyperpigmentation of the overlying skin.
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Media file 6:  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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Media file 7:  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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Media file 8:  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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Media file 9:  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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Media file 10:  High-power photomicrograph of the chronic inflammatory aggregate consists of lymphocytes and plasma cells around a ductal structure (hematoxylin-eosin, 400 X).
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REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
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• Medication
• Follow-up
• Miscellaneous
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• References

• Anaya JM, Ogawa N, Talal N. Sjogren's syndrome in childhood. J Rheumatol. Jun 1995;22(6):1152-8. [Medline].
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• Bell M, Askari A, Bookman A, et al. Sjogren's syndrome: a critical review of clinical management. J Rheumatol. Sep 1999;26(9):2051-61. [Medline].
• Cassidy JT, Petty RE, Laxer RM. Overlap syndromes. Textbook of Pediatric Rheumatology. 2005;5th edition:486-89.
• Chudwin DS, Daniels TE, Wara DW, et al. Spectrum of Sjogren syndrome in children. J Pediatr. Feb 1981;98(2):213-7. [Medline].
• Cimaz R, Casadei A, Rose C, et al. Primary Sjogren syndrome in the paediatric age: a multicentre survey. Eur J Paediatr. 2003;162 (10):661-5. [Medline].
• DeGuzman M, Fishman MA, Lewis RA, et al. Chronic neurologic disease with visual, gait and bladder problems in a male teenager. J Pediatr. 1998;132:742-47. [Medline].
• Deprettere AJ, Van Acker KJ, De Clerck LS, et al. Diagnosis of Sjogren's syndrome in children. Am J Dis Child. Nov 1988;142(11):1185-7. [Medline].
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Article Last Updated: Nov 21, 2006