Approach Considerations
Many of the symptoms associated with Sjögren syndrome can 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 adolescents. Artificial tears and conservation of natural tear flow are used in the management of keratoconjunctivitis.
Close attention must be paid to emotional and cognitive functioning of the adolescent coping with a chronic disease such as Sjögren syndrome.
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.
Discourage patients from smoking. Instruct patients to avoid windy and low-humidity environments. The family dwelling should be well humidified. Support normal school attendance and academic functioning in patients with juvenile Sjögren syndrome. [33]
Xerostomia
Means of addressing xerostomia include 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 of salivary flow with sugarless chewing gum or lozenges; and topical tissue hydration or lubrication with drinking water or artificial saliva. These measures are supportive and have only been well studied in adults, yet they may improve quality of life in patients of all ages. [34, 35]
Oral Hygiene
In Sjögren syndrome, calcium is leeched from teeth because of a reduction in saliva and as a result of the interaction between simple sugars and acidogenic bacteria. Therefore, good oral hygiene is necessary for caries control. Patients should avoid mouth rinses that contain alcohol, because they desiccate the mucosa.
Dental plaque reduction measures include twice-daily cleaning of the teeth with a toothbrush, using a fluoride-containing dentifrice, the daily use of dental floss, and increasing 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. [30]
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.
For the prevention of oral mucosal lesions such as chapped lips, use water- or lanolin-based lip moisturizers. Avoid lip products that are medicated with menthol or phenol, because they cause further drying. For traumatic erosions and ulcers, frequently hydrate and use artificial saliva or oral moisturizing agents, especially under removable oral prostheses.
For oropharyngeal candidiasis, recommend good oral hygiene, frequent oral hydration and lubrication, and nightly removal and cleaning of dental prostheses. The 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. (See the image below.)
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.
Extraglandular Manifestations
In adults, extraglandular or systemic manifestations often require immunolytic and immunomodulatory or disease-modifying antirheumatic drugs (DMARDs). No standardized immunosuppressive regimen has been established for childhood- or juvenile-onset patients. In one multicenter review, 55% of children received corticosteroids, 17.5% received nonsteroidal anti-inflammatory drugs (NSAIDS), and 12.5% received hydroxychloroquine. [10] Hydroxychloroquine was predominantly used for patients in a separate single-institution review. [17]
Consultations
The following consultations may be necessary:
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Ophthalmologist - To diagnose keratitis and uveitis using slit-lamp examination and for supportive care for sicca syndrome and inflammatory changes
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Dentist - To maintain salivary flow and to prevent caries and periodontal disease
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Rheumatologist - For the diagnosis and long-term care of adult or pediatric disease; for the evaluation of extraglandular, systemic, or overlapping autoimmune disease symptoms; and for guidance in the use of immunosuppressive medications for extraglandular manifestations
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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 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 that patients avoid dry, crunchy foods, because they are too difficult to swallow and may irritate the mucosa. Patients should also avoid spicy or acidic foods and beverages. In addition, avoiding simple carbohydrates, such as sucrose, and refined, highly processed foods, such as pastries and cookies, is important, to decrease the risk of dental caries. Alcoholic beverages and caffeinated drinks, such as coffee, tea, and cola, increase oral dryness.
If sweetener substitutes are used, monitor their 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 an increase in calories is needed because of an eating disorder, consider liquid nutritional supplements.
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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).
